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PMID	18464922 ( )
Title	Peroxisome Proliferator-Activated Receptor-gamma in Amyotrophic Lateral Sclerosis and Huntington's Disease.
Abstract	Amyotrophic lateral sclerosis (ALS) is a debilitating and one of the most common adult-onset neurodegenerative diseases with the prevalence of about 5 per 100 000 individuals. It results in the progressive loss of upper and lower motor neurons and leads to gradual muscle weakening ultimately causing paralysis and death. ALS has an obscure cause and currently no effective treatment exists. In this review, a potentially important pathway is described that can be activated by peroxisome proliferator-activated receptor-gamma (PPAR-gamma) agonists and has the ability to block the neuropathological damage caused by inflammation in ALS and possibly in other neudegenerative diseases like Huntington's disease (HD). Neuroinflammation is a common pathological feature in neurodegenerative diseases. Therefore, PPAR-gamma agonists are thought to be neuroprotective in ALS and HD. We and others have tested the neuroprotective effect of pioglitazone (Actos), a PPAR-gamma agonist, in G93A SOD1 transgenic mouse model of ALS and found significant increase in survival of G93A SOD1 mice. These findings suggest that PPAR-gamma may be an important regulator of neuroinflammation and possibly a new target for the development of therapeutic strategies for ALS. The involvement of PPAR-gamma in HD is currently under investigation, one study finds that the treatment with rosiglitazone had no protection in R6/2 transgenic mouse model of HD. PPAR-gamma coactivator-1alpha (PGC-1alpha) is a transcriptional coactivator that works together with combination of other transcription factors like PPAR-gamma in the regulation of mitochondrial biogenesis. Therefore, PPAR-gamma is a possible target for ALS and HD as it functions as transcription factor that interacts with PGC-1alpha. In this review, the role of PPAR-gamma in ALS and HD is discussed based on the current literature and hypotheses. University, New York-Presbyterian Hospital, New York, NY 10065, USA.

NOTE: Color highlight is limited to the abstract and SciMiner text-mining mode. If you see much more identified targets below from "Targets by SciMiner Summary" and "Targets by SciMiner Full list", they may have been identified from the full text.

Targets by SciMiner Summary

HUGO ID	Symbol	Target Name	#Occur	ActualStr
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	86	pgc 1 \| PGC-1 \|
9236	PPARG	peroxisome proliferator-activated receptor gamma	82	PPAR-G \|
9232	PPARA	peroxisome proliferator-activated receptor alpha	17	PPAR \| PPAR-A \| PPARs \|
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	15	SOD1 \|
19383	SOCS1	suppressor of cytokine signaling 1	7	socs 1 \| SOCS-1 \| suppressor of cytokine signaling 1 \|
4851	HTT	huntingtin	7	huntingtin \|
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	6	iNOS \| nitric oxide synthase \|
6081	INS	insulin	5	insulin \|
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	5	amp activated protein kinase \| AMPK \|
990	BCL2	B-cell CLL/lymphoma 2	4	Bcl-2 \| bcl 2 \|
14929	SIRT1	sirtuin (silent mating type information regulation 2 homolog) 1 (S. cerevisiae)	4	SIRT1 \| sirtuin 1 \|
9605	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	4	COX-2 \| cox 2 \|
7996	NRF1	nuclear respiratory factor 1	3	nuclear respiratory factor 1 \| NRF-1 \|
19391	SOCS3	suppressor of cytokine signaling 3	3	SOCS-3 \|
3796	FOS	v-fos FBJ murine osteosarcoma viral oncogene homolog	3	ap 1 \| AP-1 \| activator protein 1 \|
7176	MMP9	matrix metallopeptidase 9 (gelatinase B, 92kDa gelatinase, 92kDa type IV collagenase)	3	MMP-9 \| matrix metalloproteinase 9 \| mmp 9 \|
11741	TFAM	transcription factor A, mitochondrial	3	mitochondrial transcription factor a \| Tfam \| TFAM \|
12517	UCP1	uncoupling protein 1 (mitochondrial, proton carrier)	3	uncoupling protein 1 \| UCP-1 \|
7672	NCOR1	nuclear receptor co-repressor 1	2	N-CoR \| n cor \|
11892	TNF	tumor necrosis factor (TNF superfamily, member 2)	2	tumor necrosis factor \| TNF-A \|
7700	NDUFB5	NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa	1	NDUFB5 \|
7794	NFKB1	nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105)	1	nuclear factor kappa b \|
3471	ESRRA	estrogen-related receptor alpha	1	ERR \|
11362	STAT1	signal transducer and activator of transcription 1, 91kDa	1	STAT \|
19986	CYCS	cytochrome c, somatic	1	CYCS \|
10417	RPS27A	ribosomal protein S27a	1	ubiquitin \|
11782	TH	tyrosine hydroxylase	1	tyrosine hydroxylase \|
89	ACADM	acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain	1	ACADM \|
6541	LDHB	lactate dehydrogenase B	1	LDHB \|
10477	RXRA	retinoid X receptor, alpha	1	retinoid x receptor \|
4235	GFAP	glial fibrillary acidic protein	1	GFAP \|
9235	PPARD	peroxisome proliferator-activated receptor delta	1	PPAR-B \|
7710	NDUFS3	NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)	1	NDUFS3 \|
11919	CD40	CD40 molecule, TNF receptor superfamily member 5	1	CD40 \|
2277	COX6A1	cytochrome c oxidase subunit VIa polypeptide 1	1	COX6A1 \|

Targets by SciMiner Full list

HUGO ID	Symbol	Name	ActualStr	Score	FlankingText
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	is described that can be activated by peroxisome proliferator-activated receptor-_amp_#x003b3 PPAR-_amp_#x003b3 agonists and has the ability to block the neuropathological damage
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Therefore PPAR-_amp_#x003b3 agonists are thought to be neuroprotective in ALS and HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	have tested the neuroprotective effect of pioglitazone (Actos), Actos a PPAR-_amp_#x003b3 agonist in G93A SOD1 transgenic mouse model of ALS and
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	effect of pioglitazone (Actos), Actos a PPAR-_amp_#x003b3 agonist in G93A SOD1 transgenic mouse model of ALS and found significant increase in
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	of ALS and found significant increase in survival of G93A SOD1 mice
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	These findings suggest that PPAR-_amp_#x003b3 may be an important regulator of neuroinflammation and possibly a
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The involvement of PPAR-_amp_#x003b3 in HD is currently under investigation one study finds that
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 coactivator-1 _amp_#x003b1 (PGC-1 PGC-1 _amp_#x003b1 is a transcriptional coactivator that
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	PPAR-_amp_#x003b3 coactivator-1 _amp_#x003b1 (PGC-1 PGC-1 _amp_#x003b1 is a transcriptional coactivator that works together with combination
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	that works together with combination of other transcription factors like PPAR-_amp_#x003b3 in the regulation of mitochondrial biogenesis
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Therefore PPAR-_amp_#x003b3 is a possible target for ALS and HD as it
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	HD as it functions as transcription factor that interacts with PGC-1 _amp_#x003b1
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	In this review the role of PPAR-_amp_#x003b3 in ALS and HD is discussed based on the current
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	Peroxisome proliferator-activated receptors (PPARs) PPARs are ligand-activated transcription factors that belong to the nuclear hormone
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	that belong to the nuclear hormone receptor superfamily which includes PPAR-_amp_#x003b3 PPAR-_amp_#x003b1 and PPAR-_amp_#x003b2 / _amp_#x003b4
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR-A	2.2	belong to the nuclear hormone receptor superfamily which includes PPAR-_amp_#x003b3 PPAR-_amp_#x003b1 and PPAR-_amp_#x003b2 / _amp_#x003b4
9235	PPARD	peroxisome proliferator-activated receptor delta	PPAR-B	2.2	the nuclear hormone receptor superfamily which includes PPAR-_amp_#x003b3 PPAR-_amp_#x003b1 and PPAR-_amp_#x003b2 / _amp_#x003b4
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 is the most studied receptor and has two isoforms produced
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	isoforms produced due to alternative splicing and alternate translation initiation PPAR-_amp_#x003b3 1 and PPAR-_amp_#x003b3 2 1 2
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	to alternative splicing and alternate translation initiation PPAR-_amp_#x003b3 1 and PPAR-_amp_#x003b3 2 1 2
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	receptor from the same superfamily that forms heterodimeric complexes with PPARs in response to ligand binding
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	PPARs are ligand-dependent transcription factors that bind to specific PPREs and
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	PPARs regulate the expression of target genes in particular those associated
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR	2.5	PPAR isotypes appear to exhibit distinct patterns of tissue distribution and
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR-A	2.2	PPAR-_amp_#x003b1 is expressed in high levels in hepatocytes entrocytes and kidney
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR-A	2.2	PPAR-_amp_#x003b1 is implicated to be responsible for the peroxisome proliferator-induced pleiotropic
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR-A	2.2	PPAR-_amp_#x003b1 and _amp_#x003b4 appear primarily to stimulate oxidative lipid metabolism while
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	and _amp_#x003b4 appear primarily to stimulate oxidative lipid metabolism while PPAR-_amp_#x003b3 is principally involved in the cellular assimilation of lipids via
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Recently other functions for PPAR-_amp_#x003b3 are described such as neuroprotection in ischemia 15 and its
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 has been demonstrated to be involved in adipogenesis and differentiation
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 is shown to have a vital role in adipocyte differentiation
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Recent studies demonstrate PPAR-_amp_#x003b3 agonists to prevent inflammation and neuronal death after focal cerebral
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Thiazolidinediones (TZDs) TZDs are potent synthetic agonists of PPAR-_amp_#x003b3 shown to induce neuroprotection after cerebral ischemia by blocking inflammation
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	protective lipid-independent effects of TZDs are the anti-inflammatory capacities of PPAR-_amp_#x003b3 4
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	iNOS	2.2	of various inflammatory proteins like inducible nitric oxide synthase (iNOS), iNOS tumor necrosis factor-_amp_#x003b1 TNF-_amp_#x003b1 and matrix metalloproteinase-9 (MMP-9) MMP-9 in
11892	TNF	tumor necrosis factor (TNF superfamily, member 2)	TNF-A	1.2	like inducible nitric oxide synthase (iNOS), iNOS tumor necrosis factor-_amp_#x003b1 TNF-_amp_#x003b1 and matrix metalloproteinase-9 (MMP-9) MMP-9 in macrophages 26 and are
7176	MMP9	matrix metallopeptidase 9 (gelatinase B, 92kDa gelatinase, 92kDa type IV collagenase)	MMP-9	1.3	(iNOS), iNOS tumor necrosis factor-_amp_#x003b1 TNF-_amp_#x003b1 and matrix metalloproteinase-9 (MMP-9) MMP-9 in macrophages 26 and are beneficial in disorders such as
3796	FOS	v-fos FBJ murine osteosarcoma viral oncogene homolog	AP-1	1.0	of nuclear factor kappa B NF-_amp_#x003ba B activator protein-1 (AP-1), AP-1 in addition to signal transducers and activators of transcription (STAT)
11362	STAT1	signal transducer and activator of transcription 1, 91kDa	STAT	2.1	in addition to signal transducers and activators of transcription (STAT) STAT transcription factors by PPAR-_amp_#x003b3 31
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	transducers and activators of transcription (STAT) STAT transcription factors by PPAR-_amp_#x003b3 31
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	It is possible that PPAR-_amp_#x003b3 is involved in the reciprocal inhibition of differential transcription systems
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	an alternative mechanism suggested that a functionally distinct pool of PPAR-_amp_#x003b3 is susceptible to ligand-dependent sumoylation (covalent covalent attachment of small
7672	NCOR1	nuclear receptor co-repressor 1	N-CoR	1.3	365 leading to recruitment and stabilization of nuclear corepressor (N-CoR) N-CoR complexes at the promoters of proinflammatory genes thereby repressing them
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR	2.5	Figure 1 (a) a Structure of PPAR agonists (b) b schematic diagrams linking mechanisms of neuronal cell
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	of neuronal cell death in ALS and a representation of PPAR-_amp_#x003b3 activation
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 AND AMYOTROPHIC LATERAL SCLEROSIS
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	PPARs in particular PPAR-_amp_#x003b3 may be a major signaling pathway involved
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPARs in particular PPAR-_amp_#x003b3 may be a major signaling pathway involved in neuroinflammation in
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The activation or inactivation of PPAR-_amp_#x003b3 could provide a viable and promising approach to understand the
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 has been identified as a key regulatory factor in the
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR	2.5	key regulatory factor in the modulation of target genes with PPAR response element (PPRE) PPRE in their promoters including those encoding
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	iNOS	2.2	PPRE in their promoters including those encoding for inflammation (iNOS, iNOS NF-_amp_#x003ba B COX-2 oxidative stress and apoptosis
9605	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	COX-2	1.0	promoters including those encoding for inflammation (iNOS, iNOS NF-_amp_#x003ba B COX-2 oxidative stress and apoptosis
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Synthetic PPAR-_amp_#x003b3 agonists developed in the past 25 years that are used
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	suitable candidates and are indispensable to study the role of PPAR-_amp_#x003b3 in ALS which may potentially lead to beneficial effects in
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Previous studies have shown the protective effect of PPAR-_amp_#x003b3 agonists in many experimental models such as in experimental autoimmune
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Additionally PPAR-_amp_#x003b3 agonists are reported to be neuroprotective in tyrosine hydroxylase positive
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	have tested the neuroprotective effect of pioglitazone in transgenic G93A SOD1 mouse model of ALS and showed that pioglitazone treatment improved
11919	CD40	CD40 molecule, TNF receptor superfamily member 5	CD40	0.6	in the spinal cord as assessed by immunohistochemical staining for CD40 (microglia microglia marker and GFAP (astrocyte astrocyte marker respectively
4235	GFAP	glial fibrillary acidic protein	GFAP	0.3	assessed by immunohistochemical staining for CD40 (microglia microglia marker and GFAP (astrocyte astrocyte marker respectively
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	iNOS	2.2	Furthermore we showed that pioglitazone treatment reduced iNOS NF-_amp_#x003ba B and 3-nitotyrosine immunoreactivity in the spinal cord of
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	another study on the effect of pioglitazone treatment in G93A SOD1 transgenic mouse model of ALS 39
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	In this study PPAR-_amp_#x003b3 agonist treatment improved survival muscle strength and weight loss in
9605	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	COX-2	1.0	microglial activation as well as reduction in the expression of COX-2 and iNOS 39
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	iNOS	2.2	as well as reduction in the expression of COX-2 and iNOS 39
19383	SOCS1	suppressor of cytokine signaling 1	SOCS-1	3.5	suppressor genes suppressor of cytokine signaling 1 and 3 (SOCS-1 SOCS-1 and -3 were increased as assessed by semiquantitative RT-PCR 39
19391	SOCS3	suppressor of cytokine signaling 3	SOCS-3	1.5	suppressor genes suppressor of cytokine signaling 1 and 3 (SOCS-1 SOCS-1 and -3 were increased as assessed by semiquantitative RT-PCR 39
19383	SOCS1	suppressor of cytokine signaling 1	SOCS-1	3.5	Others have reported similar increase in SOCS-1 and -3 in response to TZDs in microglia and astrocytes
19391	SOCS3	suppressor of cytokine signaling 3	SOCS-3	1.5	Others have reported similar increase in SOCS-1 and -3 in response to TZDs in microglia and astrocytes
19383	SOCS1	suppressor of cytokine signaling 1	SOCS-1	3.5	The increase in SOCS-1 and -3 is implicated with the inhibition of Janus kinase-signal
19391	SOCS3	suppressor of cytokine signaling 3	SOCS-3	1.5	The increase in SOCS-1 and -3 is implicated with the inhibition of Janus kinase-signal
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Other studies using PPAR-_amp_#x003b3 agonists suggest that the mechanism of actions are also by
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Recently Xu and Drew demonstrated that PPAR-_amp_#x003b3 agonists suppress cytokines like IL-12 family in EAE an experimental
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	These studies provide evidence that PPAR-_amp_#x003b3 agonist responses are originating from activated glial cells in central
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR	2.5	PPAR agonists are shown to modulate microglia and astrocytes in central
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	showed reduction in gliosis which is another experimental evidence that PPAR-_amp_#x003b3 acts on glial cells in CNS 38
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The action of PPAR-_amp_#x003b3 in neuronal cells needs to be studied
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The preliminary reports on the neuroprotective role of PPAR-_amp_#x003b3 agonist in transgenic mouse model of ALS and other experimental
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	of studies to understand the mechanism and molecular details of PPARs and their role in protecting motor neurons from inflammatory damages
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The mechanisms of how PPAR-_amp_#x003b3 agonists induce neuroprotection by blocking neuroinflammation is not fully understood
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	fully understood and further information on the molecular details of PPAR-_amp_#x003b3 in neuroinflammatory pathways will provide crucial insights on the role
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	neuroinflammatory pathways will provide crucial insights on the role of PPAR-_amp_#x003b3 in ALS and other neurodegenerative diseases
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	of mitochondrial dysfunction in FALS-SOD1 it is hypothesized that mutant SOD1 may directly damage mitochondrial function and integrity
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	Several studies have shown that transgenic mice overexpressing human G93A SOD1 that display most of the ALS symptoms and pathologies have
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	we and others have shown that wild type and mutant SOD1 are found within mitochondrion which was known to be a
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	How SOD1 is interacting with mitochondria is unclear and it is being
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	The toxic action of mutant SOD1 in and out of mitochondria could be partly explained as
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	(i) i Mutant but not wild type SOD1 binds to heat shock proteins causing an inhibition of chaperon
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	Both mutant and wild type SOD1 bind to antiapoptotic protein Bcl-2 on the outer mitochondrial membrane
990	BCL2	B-cell CLL/lymphoma 2	Bcl-2	1.8	Both mutant and wild type SOD1 bind to antiapoptotic protein Bcl-2 on the outer mitochondrial membrane blocking its antiapoptotic activity 42
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	(ii) ii The presence of mutant SOD1 in the mitochondria leads to formation of SOD1 aggregates entrapping
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	of mutant SOD1 in the mitochondria leads to formation of SOD1 aggregates entrapping Bcl-2 blocking protein importation to mitochondria which may
990	BCL2	B-cell CLL/lymphoma 2	Bcl-2	1.8	in the mitochondria leads to formation of SOD1 aggregates entrapping Bcl-2 blocking protein importation to mitochondria which may trigger neuronal cell
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Since PGC-1 _amp_#x003b1 is known to coordinate mitochondrial biogenesis and regulates mitochondrial
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	and regulates mitochondrial function it is possible to predict that PGC-1 _amp_#x003b1 could play an important role in ALS
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Impairment of PGC-1 _amp_#x003b1 could contribute to mitochondrial dysfunction in ALS
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	date there is no published data on the role of PGC-1 _amp_#x003b1 or its expression in the transgenic mouse model of
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	genes in ALS that some of them fit in the PGC-1 _amp_#x003b1 target genes category 29 48 suggesting that there may
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	48 suggesting that there may be a prominent role for PGC-1 _amp_#x003b1 translational machinery in ALS
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Since PGC-1 _amp_#x003b1 is a PPAR-_amp_#x003b3 coactivator it is possible that PPAR-_amp_#x003b3
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Since PGC-1 _amp_#x003b1 is a PPAR-_amp_#x003b3 coactivator it is possible that PPAR-_amp_#x003b3 agonists may be able
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PGC-1 _amp_#x003b1 is a PPAR-_amp_#x003b3 coactivator it is possible that PPAR-_amp_#x003b3 agonists may be able to activate PGC-1 _amp_#x003b1 and also
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	is possible that PPAR-_amp_#x003b3 agonists may be able to activate PGC-1 _amp_#x003b1 and also the PGC-1 _amp_#x003b1 target genes
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	may be able to activate PGC-1 _amp_#x003b1 and also the PGC-1 _amp_#x003b1 target genes
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Like in HD a reduction of PGC-1 _amp_#x003b1 and its target genes expression is attributed to mutant
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	target genes expression is attributed to mutant huntingtin similarly mutant SOD1 could impair PGC-1 _amp_#x003b1 and expression of its target genes
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	is attributed to mutant huntingtin similarly mutant SOD1 could impair PGC-1 _amp_#x003b1 and expression of its target genes in ALS
11179	SOD1	superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))	SOD1	0.5	Whether mutant SOD1 can impair PPAR-_amp_#x003b3 is yet to be determined
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Whether mutant SOD1 can impair PPAR-_amp_#x003b3 is yet to be determined
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Future studies on PGC-1 _amp_#x003b1 and PPAR-_amp_#x003b3 in ALS patients and transgenic mice will
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Future studies on PGC-1 _amp_#x003b1 and PPAR-_amp_#x003b3 in ALS patients and transgenic mice will shed some lights
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 AND HUNTINGTON'S DISEASE
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Recent reports show that mutant huntingtin interferes with transcriptional PPAR-_amp_#x003b3 coactivator-1 _amp_#x003b1 (PGC-1 PGC-1 _amp_#x003b1 causing impairment on its function
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	that mutant huntingtin interferes with transcriptional PPAR-_amp_#x003b3 coactivator-1 _amp_#x003b1 (PGC-1 PGC-1 _amp_#x003b1 causing impairment on its function in HD suggesting that
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	that mutant huntingtin plays a role in the dysregulation of PGC-1 _amp_#x003b1 -mediated transcription and activity impairing mitochondrial function and leading
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Weydt et al found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM
19986	CYCS	cytochrome c, somatic	CYCS	0.3	et al found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in
2277	COX6A1	cytochrome c oxidase subunit VIa polypeptide 1	COX6A1	0.3	al found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in HD
11741	TFAM	transcription factor A, mitochondrial	TFAM	0.9	PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in HD patient and mouse
7710	NDUFS3	NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)	NDUFS3	0.3	Weydt et al found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression
7700	NDUFB5	NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa	NDUFB5	0.4	found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in HD patient
89	ACADM	acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain	ACADM	0.3	that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in HD patient and
6541	LDHB	lactate dehydrogenase B	LDHB	0.1	target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM and LDHB had reduced expression in HD patient and mouse striatum 27
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	An interesting finding was that the PGC-1 _amp_#x003b1 and uncoupling protein 1 (UCP-1) UCP-1 circuit was found
12517	UCP1	uncoupling protein 1 (mitochondrial, proton carrier)	UCP-1	1.4	was that the PGC-1 _amp_#x003b1 and uncoupling protein 1 (UCP-1) UCP-1 circuit was found to be disrupted in the brown adipose
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	In HD and wild type mice challenged with cold PGC-1 _amp_#x003b1 expression increased but in HD mice UCP-1 expression was
12517	UCP1	uncoupling protein 1 (mitochondrial, proton carrier)	UCP-1	1.4	with cold PGC-1 _amp_#x003b1 expression increased but in HD mice UCP-1 expression was not upregulated
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	However they showed that PGC-1 _amp_#x003b1 expression is decreased in the striatum of human HD
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPAR-A	2.2	They also examined the expression of unclear hormone receptors PPAR-_amp_#x003b1 RXR-_amp_#x003b1 and transcription factors (NRF-1) NRF-1 that known to rely
7996	NRF1	nuclear respiratory factor 1	NRF-1	1.2	of unclear hormone receptors PPAR-_amp_#x003b1 RXR-_amp_#x003b1 and transcription factors (NRF-1) NRF-1 that known to rely upon PGC-1 _amp_#x003b1 for target gene
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	and transcription factors (NRF-1) NRF-1 that known to rely upon PGC-1 _amp_#x003b1 for target gene activation these genes were upregulated suggesting
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	activation these genes were upregulated suggesting possible compensatory upregulation of PGC-1 _amp_#x003b1 -dependent transcription factors in human HD caudate
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	et al studies provide further support that the reduction of PGC-1 _amp_#x003b1 and its target genes in HD striatum are caused
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	and R.E Hughes that their yeast two-hybrid screen identified that PPAR-_amp_#x003b3 is a huntingtin interactor and the interaction was validated for
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	validated for its biological significance by demonstrating an effect of PPAR-_amp_#x003b3 dosage upon HD neurodegeneration in the fly eye 27
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	iNOS	2.2	Increased levels of iNOS in HD 59 elevated oxidative damage products such as malondialdehyde
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	must be explored in order to understand the role of PPAR-_amp_#x003b3 and to identify new therapeutic targets for HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Rosiglitazone (a a PPAR-_amp_#x003b3 agonist that induces sensitization to insulin was tested in R6/2
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	2 mice it also provided data for the role of PPAR-_amp_#x003b3 in R6/2 R6 2 mice
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	AMPK	3.2	hepatic glucose synthesis 65 activation of AMP-activated protein kinase (AMPK, AMPK an enzyme involved in glucose and fatty acid metabolism 66
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	AMPK	3.2	Activation of AMPK is associated with mitochondrial proliferation and biogenesis 69
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Rosiglitazone was used as PPAR-_amp_#x003b3 agonist in R6/2 R6 2 mice which could be used
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	be used as the bases to test the role of PPAR-_amp_#x003b3 in HD glibenclamide and metformin were used to treat atypical
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	AMPK	3.2	Metformin treatment in R6/2 R6 2 mice increased brain AMPK phosphorylation 62 although this needs to be confirmed
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	AMPK	3.2	Activation of AMPK leads to reduction in ATP-consuming processes and facilitate ATP-generating cellular
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	be the synergistic effect from several pathways including regulation of PGC-1 _amp_#x003b1 activity through its direct activation of AMPA kinase
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Metformin does not belong to any class of PPAR-_amp_#x003b3 agonists although it is an antidiabetic for type-2 diabetes and
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	PGC-1 _amp_#x003b1 has been implicated in mitochondrial biogenesis through its ability
7996	NRF1	nuclear respiratory factor 1	NRF-1	1.2	number of genes such as nuclear respiratory factor-1 -2 (NRF-1,-2), NRF-1 -2 estrogen related receptor _amp_#x003b1 (ERR ERR _amp_#x003b1 and mitochondrial
3471	ESRRA	estrogen-related receptor alpha	ERR	0.3	factor-1 -2 (NRF-1,-2), NRF-1 -2 estrogen related receptor _amp_#x003b1 (ERR ERR _amp_#x003b1 and mitochondrial transcription factor A (Tfam) Tfam 70
11741	TFAM	transcription factor A, mitochondrial	Tfam	0.9	_amp_#x003b1 (ERR ERR _amp_#x003b1 and mitochondrial transcription factor A (Tfam) Tfam 70
14929	SIRT1	sirtuin (silent mating type information regulation 2 homolog) 1 (S. cerevisiae)	SIRT1	0.3	Resveratrol has been shown to activate sirtuin 1 (SIRT1) SIRT1 and results in PPAR-_amp_#x003b3 -mediated transcriptional repression inhibition of adipogenesis
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	shown to activate sirtuin 1 (SIRT1) SIRT1 and results in PPAR-_amp_#x003b3 -mediated transcriptional repression inhibition of adipogenesis enhanced lipolysis and the
14929	SIRT1	sirtuin (silent mating type information regulation 2 homolog) 1 (S. cerevisiae)	SIRT1	0.3	Activated SIRT1 leads to deactylation of PGC-1 _amp_#x003b1 resulting in an activation
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Activated SIRT1 leads to deactylation of PGC-1 _amp_#x003b1 resulting in an activation of PGC-1 _amp_#x003b1 73
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	to deactylation of PGC-1 _amp_#x003b1 resulting in an activation of PGC-1 _amp_#x003b1 73
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	By deacetylating PGC-1 _amp_#x003b1 SIRT1 represses glycolysis increase hepatic glucose output and modulates
14929	SIRT1	sirtuin (silent mating type information regulation 2 homolog) 1 (S. cerevisiae)	SIRT1	0.3	By deacetylating PGC-1 _amp_#x003b1 SIRT1 represses glycolysis increase hepatic glucose output and modulates mitochondrial function
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	PGC-1 _amp_#x003b1 is known as master regulator of mitochondrial biogenesis and
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Although PPAR-_amp_#x003b3 agonist treatments in R6/2 R6 2 failed it is premature
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	is premature to conclude that there is no role for PPARs in HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	in other models of HD are required to examine other PPAR-_amp_#x003b3 agonists
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Moreover the effect of PPAR-_amp_#x003b3 agonists on the expression and activation of PGC-1 _amp_#x003b1 in
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	effect of PPAR-_amp_#x003b3 agonists on the expression and activation of PGC-1 _amp_#x003b1 in cell culture models of HD may provide preliminary
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	rationale for that is based on the increasing evidence that PGC-1 _amp_#x003b1 expression which is downregulated in patients with Huntington's disease
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Thiazolidiones and rexinoids induce PGC-1 _amp_#x003b1 gene transcription in brown and white adipocytes 75
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	oxidation and adaptive thermoregulation then it can be predicted that PPAR-_amp_#x003b3 agonists could help HD mice to maintain thermoregulatory function when
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Based on the studies on PGC-1 _amp_#x003b1 knockout mice that shown to have neurodegenerative lesions particularly
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	shown to have neurodegenerative lesions particularly in striatum suggest that PGC-1 _amp_#x003b1 may have an important function in neurons 76
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	However the neurodegenerative lesions in PGC-1 _amp_#x003b1 knockout mice do not mimic lesions in HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	The role of PPAR-_amp_#x003b3 in ALS AD and Parkinson's disease are backed with evidence
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	with evidence 19 20 38 39 while the role of PPAR-_amp_#x003b3 in HD lacks critical evidence and needs to be studied
9232	PPARA	peroxisome proliferator-activated receptor alpha	PPARs	2.2	model of HD could shed light on the role of PPARs in HD
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	mitochondrial biogenesis impairment in HD and potential neuroprotective role of PGC-1 _amp_#x003b1 in HD PPAR-_amp_#x003b3 desperately seeking further attention and these
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	HD and potential neuroprotective role of PGC-1 _amp_#x003b1 in HD PPAR-_amp_#x003b3 desperately seeking further attention and these types of studies could
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	of studies could provide essential data on the role of PPAR-_amp_#x003b3 in HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Studies in patients treated with PPAR-_amp_#x003b3 agonists indicate that the reduction of insulin resistance is resulted
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	reduction of insulin resistance is resulted from the activation of PPAR-_amp_#x003b3 78
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	PPAR-_amp_#x003b3 's natural coactivator is PGC-1 _amp_#x003b1
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	PPAR-_amp_#x003b3 's natural coactivator is PGC-1 _amp_#x003b1
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	TZDs can mimic the effect of PGC-1 _amp_#x003b1 on PPAR-_amp_#x003b3
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	TZDs can mimic the effect of PGC-1 _amp_#x003b1 on PPAR-_amp_#x003b3
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	If PGC-1 _amp_#x003b1 levels reduces or become inactivated by acetylation then the
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	reduces or become inactivated by acetylation then the activity of PPAR-_amp_#x003b3 could be affected
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	In this review we highlighted the role of PPAR-_amp_#x003b3 in neurodegenerative diseases in particular in a mouse model of
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	two independent studies provides strong indication for the involvement of PPAR-_amp_#x003b3 in ALS
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	Whether PPAR-_amp_#x003b3 is involved in HD remains to be clarified as one
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	the treatment of R6/2 R6 2 mice with rosiglitazone another PPAR-_amp_#x003b3 agonist had no beneficial effect
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	In the future we will explore the mechanisms by which PPAR-_amp_#x003b3 agonists produce neuroprotection in a mouse model of ALS and
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	neuroprotection in a mouse model of ALS and test whether PPAR-_amp_#x003b3 has a role in HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	be of great interest to determine whether the effect of PPAR-_amp_#x003b3 is powered by glial or neuronal cells or both in
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	also be of great interest to determine the effect of PPAR-_amp_#x003b3 agonist on muscles in ALS and HD mouse models
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	cell culture studies are necessary in determining the role of PPAR-_amp_#x003b3 in ALS and HD
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	it would be very informative to test the effect of PPAR-_amp_#x003b3 agonists on HD mouse models for their effect in thermoregulation
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	The activation of PGC-1 _amp_#x003b1 in HD mouse models or overexpression of PGC-1 _amp_#x003b1
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	of PGC-1 _amp_#x003b1 in HD mouse models or overexpression of PGC-1 _amp_#x003b1 in HD mouse models show efficacy in blockage of
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	are confirmed then there is bonafide evidence that activation of PGC-1 _amp_#x003b1 could be a great therapeutic strategy for HD
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	The lack of report on the role of PGC-1 _amp_#x003b1 in ALS is a limiting step on the hypothesis
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	in ALS is a limiting step on the hypothesis that PGC-1 _amp_#x003b1 could be a target of investigation or therapeutic for
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	Mitochondria have been implicated in ALS and PGC-1 _amp_#x003b1 has possible role in mitochondrial biogenesis therefore it would
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	therefore it would be informative to examine mitochondrial abnormalities and PGC-1 _amp_#x003b1 in ALS
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	However since PPAR-_amp_#x003b3 agonist shown to activate PGC-1 _amp_#x003b1 therefore there is an
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	However since PPAR-_amp_#x003b3 agonist shown to activate PGC-1 _amp_#x003b1 therefore there is an indirect possibility that PGC-1 _amp_#x003b1
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	PGC-1	1.2	activate PGC-1 _amp_#x003b1 therefore there is an indirect possibility that PGC-1 _amp_#x003b1 in connection with PPAR-_amp_#x003b3 could play some role in
9236	PPARG	peroxisome proliferator-activated receptor gamma	PPAR-G	2.2	is an indirect possibility that PGC-1 _amp_#x003b1 in connection with PPAR-_amp_#x003b3 could play some role in ALS
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	ppar _amp_#x003b3; coactivator 1 _amp_#x003b1; pgc 1 _amp_#x003b1; is a transcriptional coactivator that works together with combination of other transcription factors like ppar _amp_#x003b3; in the regulation of mitochondrial biogenesis.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	therefore ppar _amp_#x003b3; is a possible target for als and hd as it functions as transcription factor that interacts with pgc 1 _amp_#x003b1; .
10477	RXRA	retinoid X receptor, alpha	retinoid x receptor	1.0	another ligand activated transcription factor is retinoid x receptor from the same superfamily that forms heterodimeric complexes with ppars in response to ligand binding.
7176	MMP9	matrix metallopeptidase 9 (gelatinase B, 92kDa gelatinase, 92kDa type IV collagenase)	mmp 9	1.0	tzds inhibit the expression of various inflammatory proteins like inducible nitric oxide synthase inos tumor necrosis factor _amp_#x003b1; tnf _amp_#x003b1; and matrix metalloproteinase 9 mmp 9 in macrophages [ 26 ] and are beneficial in disorders such as inflammatory bowel disease [ 27 ].
7176	MMP9	matrix metallopeptidase 9 (gelatinase B, 92kDa gelatinase, 92kDa type IV collagenase)	matrix metalloproteinase 9	1.0	tzds inhibit the expression of various inflammatory proteins like inducible nitric oxide synthase inos tumor necrosis factor _amp_#x003b1; tnf _amp_#x003b1; and matrix metalloproteinase 9 mmp 9 in macrophages [ 26 ] and are beneficial in disorders such as inflammatory bowel disease [ 27 ].
11892	TNF	tumor necrosis factor (TNF superfamily, member 2)	tumor necrosis factor	1.0	tzds inhibit the expression of various inflammatory proteins like inducible nitric oxide synthase inos tumor necrosis factor _amp_#x003b1; tnf _amp_#x003b1; and matrix metalloproteinase 9 mmp 9 in macrophages [ 26 ] and are beneficial in disorders such as inflammatory bowel disease [ 27 ].
7873	NOS2A	nitric oxide synthase 2A (inducible, hepatocytes)	nitric oxide synthase	1.0	tzds inhibit the expression of various inflammatory proteins like inducible nitric oxide synthase inos tumor necrosis factor _amp_#x003b1; tnf _amp_#x003b1; and matrix metalloproteinase 9 mmp 9 in macrophages [ 26 ] and are beneficial in disorders such as inflammatory bowel disease [ 27 ].
3796	FOS	v-fos FBJ murine osteosarcoma viral oncogene homolog	activator protein 1	1.0	several anti inflammatory mechanisms have been suggested including inhibition of nuclear factor kappa b nf _amp_#x003ba; b activator protein 1 ap 1 in addition to signal transducers and activators of transcription stat transcription factors by ppar _amp_#x003b3; [ 31 ].
7794	NFKB1	nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105)	nuclear factor kappa b	1.0	several anti inflammatory mechanisms have been suggested including inhibition of nuclear factor kappa b nf _amp_#x003ba; b activator protein 1 ap 1 in addition to signal transducers and activators of transcription stat transcription factors by ppar _amp_#x003b3; [ 31 ].
3796	FOS	v-fos FBJ murine osteosarcoma viral oncogene homolog	ap 1	1.0	several anti inflammatory mechanisms have been suggested including inhibition of nuclear factor kappa b nf _amp_#x003ba; b activator protein 1 ap 1 in addition to signal transducers and activators of transcription stat transcription factors by ppar _amp_#x003b3; [ 31 ].
7672	NCOR1	nuclear receptor co-repressor 1	n cor	1.0	of ppar _amp_#x003b3; is susceptible to ligand dependent sumoylation covalent attachment of small ubiquitin like modifier at lysine 365 leading to recruitment and stabilization of nuclear corepressor n cor complexes at the promoters of proinflammatory genes thereby repressing them [ 32 ].
10417	RPS27A	ribosomal protein S27a	ubiquitin	1.0	recently an alternative mechanism suggested that a functionally distinct pool of ppar _amp_#x003b3; is susceptible to ligand dependent sumoylation covalent attachment of small ubiquitin like modifier at lysine 365 leading to recruitment and stabilization of nuclear corepressor n cor complexes at the promoters of proinflammatory genes thereby repressing them [ 32 ].
9605	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	cox 2	1.0	; has been identified as a key regulatory factor in the modulation of target genes with ppar response element ppre in their promoters including those encoding for inflammation inos nf _amp_#x003ba; b cox 2 oxidative stress and apoptosis.
11782	TH	tyrosine hydroxylase	tyrosine hydroxylase	1.0	additionally ppar _amp_#x003b3; agonists are reported to be neuroprotective in tyrosine hydroxylase positive neurons in substantia nigra when exposed to 1 methyl 4 phenyl 1 2 3 6 tetrahydropyridine mptp [ 18 19 ].
9605	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	cox 2	1.0	they also showed significant reduction in microglial activation as well as reduction in the expression of cox 2 and inos [ 39 ].
19383	SOCS1	suppressor of cytokine signaling 1	socs 1	1.0	ulation of proinflammatory markers by pioglitazone were reported by sch_amp_#x000fc;tz et al. which suggests that mrna levels of two cytokine suppressor genes suppressor of cytokine signaling 1 and 3 socs 1 and 3 were increased as assessed by semiquantitative rt pcr [ 39 ].
19383	SOCS1	suppressor of cytokine signaling 1	suppressor of cytokine signaling 1	1.0	further evidence in the modulation of proinflammatory markers by pioglitazone were reported by sch_amp_#x000fc;tz et al. which suggests that mrna levels of two cytokine suppressor genes suppressor of cytokine signaling 1 and 3 socs 1 and 3 were increased as assessed by semiquantitative rt pcr [ 39 ].
19383	SOCS1	suppressor of cytokine signaling 1	socs 1	1.0	others have reported similar increase in socs 1 and 3 in response to tzds in microglia and astrocytes in vitro [ 40 ].
19383	SOCS1	suppressor of cytokine signaling 1	socs 1	1.0	the increase in socs 1 and 3 is implicated with the inhibition of janus kinase signal transducer and activator of transcription jak stat in inflammatory signal transduction.
990	BCL2	B-cell CLL/lymphoma 2	bcl 2	1.0	both mutant and wild type sod1 bind to antiapoptotic protein bcl 2 on the outer mitochondrial membrane blocking its antiapoptotic activity [ 42 ].
990	BCL2	B-cell CLL/lymphoma 2	bcl 2	1.0	ii the presence of mutant sod1 in the mitochondria leads to formation of sod1 aggregates entrapping bcl 2 blocking protein importation to mitochondria which may trigger neuronal cell death due to mitochondrial dysfunction [ 42 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	since pgc 1 _amp_#x003b1; is known to coordinate mitochondrial biogenesis and regulates mitochondrial function it is possible to predict that pgc 1 _amp_#x003b1; could play an important role in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	impairment of pgc 1 _amp_#x003b1; could contribute to mitochondrial dysfunction in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	to date there is no published data on the role of pgc 1 _amp_#x003b1; or its expression in the transgenic mouse model of als or human als postmortem tissues.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	however there are reports on the altered or impaired expression of genes in als that some of them fit in the pgc 1 _amp_#x003b1; target genes category [ 29 48 ] suggesting that there may be a prominent role for pgc 1 _amp_#x003b1; translational machinery in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	_amp_#x003b1; target genes category [ 29 48 ] suggesting that there may be a prominent role for pgc 1 _amp_#x003b1; translational machinery in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	since pgc 1 _amp_#x003b1; is a ppar _amp_#x003b3; coactivator it is possible that ppar _amp_#x003b3; agonists may be able to activate pgc 1 _amp_#x003b1; and also the pgc 1 _amp_#x003b1; target genes.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	like in hd a reduction of pgc 1 _amp_#x003b1; and its target genes expression is attributed to mutant huntingtin similarly mutant sod1 could impair pgc 1 _amp_#x003b1; and expression of its target genes in als.
4851	HTT	huntingtin	huntingtin	1.0	like in hd a reduction of pgc 1 _amp_#x003b1; and its target genes expression is attributed to mutant huntingtin similarly mutant sod1 could impair pgc 1 _amp_#x003b1; and expression of its target genes in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	future studies on pgc 1 _amp_#x003b1; and ppar _amp_#x003b3; in als patients and transgenic mice will shed some lights on these pathways in disease development.
4851	HTT	huntingtin	huntingtin	1.0	the etiology of hd is shown to be the unstable cag repeat expansion in the huntingtin gene on chromosome 4 resulting in polyglutamine expansion in huntingtin protein.
4851	HTT	huntingtin	huntingtin	1.0	the polyglutamine expansion causes the aggregation of huntingtin protein and formation of neuronal inclusion bodies as reviewed by ortega et al. [ 50 ].
4851	HTT	huntingtin	huntingtin	1.0	ively been studied and in spite of some new and novel discoveries and hypotheses it is not fully understood how mitochondrial dysfunction and oxidative stress and expansion of unstable cag repeats in huntingtin gene cause hd.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	recent reports show that mutant huntingtin interferes with transcriptional ppar _amp_#x003b3; coactivator 1 _amp_#x003b1; pgc 1 _amp_#x003b1; causing impairment on its function in hd suggesting that mutant huntingtin plays a role in the dysregulation of pgc 1 _amp_#x003b1; mediated transcription and activity impairing mitocho
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	_amp_#x003b1; causing impairment on its function in hd suggesting that mutant huntingtin plays a role in the dysregulation of pgc 1 _amp_#x003b1; mediated transcription and activity impairing mitochondrial function and leading to hd pathogenesis [ 56 _amp_#x02013; 58 ].
4851	HTT	huntingtin	huntingtin	1.0	recent reports show that mutant huntingtin interferes with transcriptional ppar _amp_#x003b3; coactivator 1 _amp_#x003b1; pgc 1 _amp_#x003b1; causing impairment on its function in hd suggesting that mutant huntingtin plays a role in the dysre
4851	HTT	huntingtin	huntingtin	1.0	interferes with transcriptional ppar _amp_#x003b3; coactivator 1 _amp_#x003b1; pgc 1 _amp_#x003b1; causing impairment on its function in hd suggesting that mutant huntingtin plays a role in the dysregulation of pgc 1 _amp_#x003b1; mediated transcription and activity impairing mitochondrial function and leading to hd pathogenesis [ 56 _amp_#x02013; 58 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	weydt et al. found that pgc 1 _amp_#x003b1; target genes ndufs3 cycs cox6a1 ndufb5 acadm tfam and ldhb had reduced expression in hd patient and mouse striatum [ 27 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	an interesting finding was that the pgc 1 _amp_#x003b1; and uncoupling protein 1 ucp 1 circuit was found to be disrupted in the brown adipose tissue bat of hd transgenic mice.
12517	UCP1	uncoupling protein 1 (mitochondrial, proton carrier)	uncoupling protein 1	1.0	an interesting finding was that the pgc 1 _amp_#x003b1; and uncoupling protein 1 ucp 1 circuit was found to be disrupted in the brown adipose tissue bat of hd transgenic mice.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	in hd and wild type mice challenged with cold pgc 1 _amp_#x003b1; expression increased but in hd mice ucp 1 expression was not upregulated.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	however they showed that pgc 1 _amp_#x003b1; expression is decreased in the striatum of human hd.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	they also examined the expression of unclear hormone receptors ppar _amp_#x003b1; rxr _amp_#x003b1; and transcription factors nrf 1 that known to rely upon pgc 1 _amp_#x003b1; for target gene activation these genes were upregulated suggesting possible compensatory upregulation of pgc 1 _amp_#x003b1; dependent transcription factors in human hd caudate.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	_amp_#x003b1; for target gene activation these genes were upregulated suggesting possible compensatory upregulation of pgc 1 _amp_#x003b1; dependent transcription factors in human hd caudate.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	weydt et al. and cui et al. studies provide further support that the reduction of pgc 1 _amp_#x003b1; and its target genes in hd striatum are caused by mutant huntingtin.
4851	HTT	huntingtin	huntingtin	1.0	weydt et al. stated from personal communication with j. boats and r.e. hughes that their yeast two hybrid screen identified that ppar _amp_#x003b3; is a huntingtin interactor and the interaction was validated for its biological significance by demonstrating an effect of ppar _amp_#x003b3; dosage upon hd neurodegeneration in the fly eye [ 27 ].
6081	INS	insulin	insulin	1.0	rosiglitazone a ppar _amp_#x003b3; agonist that induces sensitization to insulin was tested in r6/2 transgenic mouse model of hd for the treatment of atypical diabetes in these mice [ 61 ].
6081	INS	insulin	insulin	1.0	the effect of glibenclamide a sulfonylurea that depolarizes pancreatic beta cells by blocking atp sensitive potassium channels to induce exocytosis of insulin leading to increase in insulin levels was also tested in r6/2 mice.
9376	PRKAA1	protein kinase, AMP-activated, alpha 1 catalytic subunit	amp activated protein kinase	1.0	metformin has numerous effects on metabolism including insulin sensitization [ 63 ] increased glucose uptake [ 64 ] decrease hepatic glucose synthesis [ 65 ] activation of amp activated protein kinase ampk an enzyme involved in glucose and fatty acid metabolism [ 66 ] and mitochondrial inhibition [ 67 68 ].
6081	INS	insulin	insulin	1.0	metformin has numerous effects on metabolism including insulin sensitization [ 63 ] increased glucose uptake [ 64 ] decrease hepatic glucose synthesis [ 65 ] activation of amp activated protein kinase ampk an enzyme involved in glucose and fatty acid metabolism
6081	INS	insulin	insulin	1.0	metformin was also considered to be effective in r6/2 mice because of its ability to sensitize insulin which leads to facilitation of glucose utilization.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	the protective effect of metformin in r6/2 mice could be the synergistic effect from several pathways including regulation of pgc 1 _amp_#x003b1; activity through its direct activation of ampa kinase.
11741	TFAM	transcription factor A, mitochondrial	mitochondrial transcription factor a	1.0	been implicated in mitochondrial biogenesis through its ability to control number of genes such as nuclear respiratory factor 1 2 nrf 1 2 estrogen related receptor _amp_#x003b1; err _amp_#x003b1; and mitochondrial transcription factor a tfam [ 70 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	pgc 1 _amp_#x003b1; has been implicated in mitochondrial biogenesis through its ability to control number of genes such as nuclear respiratory factor 1 2 nrf 1 2 estrogen related receptor _amp_#x003b1; err
7996	NRF1	nuclear respiratory factor 1	nuclear respiratory factor 1	1.0	pgc 1 _amp_#x003b1; has been implicated in mitochondrial biogenesis through its ability to control number of genes such as nuclear respiratory factor 1 2 nrf 1 2 estrogen related receptor _amp_#x003b1; err _amp_#x003b1; and mitochondrial transcription factor a tfam [ 70 ].
14929	SIRT1	sirtuin (silent mating type information regulation 2 homolog) 1 (S. cerevisiae)	sirtuin 1	1.0	resveratrol has been shown to activate sirtuin 1 sirt1 and results in ppar _amp_#x003b3; mediated transcriptional repression inhibition of adipogenesis enhanced lipolysis and the release of free fatty acids [ 72 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	activated sirt1 leads to deactylation of pgc 1 _amp_#x003b1; resulting in an activation of pgc 1 _amp_#x003b1; [ 73 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	by deacetylating pgc 1 _amp_#x003b1; sirt1 represses glycolysis increase hepatic glucose output and modulates mitochondrial function and biogenesis [ 73 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	pgc 1 _amp_#x003b1; is known as master regulator of mitochondrial biogenesis and is shown to modulate a number of metabolically relevant transcription factors that collectively help in mitochondrial biogen
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	moreover the effect of ppar _amp_#x003b3; agonists on the expression and activation of pgc 1 _amp_#x003b1; in cell culture models of hd may provide preliminary data to plan full scale studies in animal models of hd.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	the rationale for that is based on the increasing evidence that pgc 1 _amp_#x003b1; expression which is downregulated in patients with huntington's disease and in several animal models of this neurodegenerative disorder [ 70 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	thiazolidiones and rexinoids induce pgc 1 _amp_#x003b1; gene transcription in brown and white adipocytes [ 75 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	based on the studies on pgc 1 _amp_#x003b1; knockout mice that shown to have neurodegenerative lesions particularly in striatum suggest that pgc 1 _amp_#x003b1; may have an important function in neurons [ 76 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	however the neurodegenerative lesions in pgc 1 _amp_#x003b1; knockout mice do not mimic lesions in hd.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	considering recent results on thermoregulation and mitochondrial biogenesis impairment in hd and potential neuroprotective role of pgc 1 _amp_#x003b1; in hd ppar _amp_#x003b3; desperately seeking further attention and these types of studies could provide essential data on the role of ppar _amp_#x003b3; in hd.
6081	INS	insulin	insulin	1.0	studies in patients treated with ppar _amp_#x003b3; agonists indicate that the reduction of insulin resistance is resulted from the activation of ppar _amp_#x003b3; [ 78 ].
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	ppar _amp_#x003b3; 's natural coactivator is pgc 1 _amp_#x003b1; .
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	tzds can mimic the effect of pgc 1 _amp_#x003b1; on ppar _amp_#x003b3; .
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	if pgc 1 _amp_#x003b1; levels reduces or become inactivated by acetylation then the activity of ppar _amp_#x003b3; could be affected.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	the activation of pgc 1 _amp_#x003b1; in hd mouse models or overexpression of pgc 1 _amp_#x003b1; in hd mouse models show efficacy in blockage of neuronal death and lead to improvement in behavioral phenotypes and increase in survival in several hd mouse models.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	if these are confirmed then there is bonafide evidence that activation of pgc 1 _amp_#x003b1; could be a great therapeutic strategy for hd.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	the lack of report on the role of pgc 1 _amp_#x003b1; in als is a limiting step on the hypothesis that pgc 1 _amp_#x003b1; could be a target of investigation or therapeutic for als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	mitochondria have been implicated in als and pgc 1 _amp_#x003b1; has possible role in mitochondrial biogenesis therefore it would be informative to examine mitochondrial abnormalities and pgc 1 _amp_#x003b1; in als.
9237	PPARGC1A	peroxisome proliferator-activated receptor gamma, coactivator 1 alpha	pgc 1	1.0	however since ppar _amp_#x003b3; agonist shown to activate pgc 1 _amp_#x003b1; therefore there is an indirect possibility that pgc 1 _amp_#x003b1; in connection with ppar _amp_#x003b3; could play some role in als.