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| 18464922 | PGC1 | 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 | |  |
| 18464922 | PGC1 | PGC-1 | 1.2 | HD as it functions as transcription factor that interacts with PGC-1 _amp_#x003b1 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Since PGC-1 _amp_#x003b1 is known to coordinate mitochondrial biogenesis and regulates mitochondrial | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Impairment of PGC-1 _amp_#x003b1 could contribute to mitochondrial dysfunction in ALS | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | 48 suggesting that there may be a prominent role for PGC-1 _amp_#x003b1 translational machinery in ALS | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Since PGC-1 _amp_#x003b1 is a PPAR-_amp_#x003b3 coactivator it is possible that PPAR-_amp_#x003b3 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | may be able to activate PGC-1 _amp_#x003b1 and also the PGC-1 _amp_#x003b1 target genes | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Like in HD a reduction of PGC-1 _amp_#x003b1 and its target genes expression is attributed to mutant | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Future studies on PGC-1 _amp_#x003b1 and PPAR-_amp_#x003b3 in ALS patients and transgenic mice will | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Weydt et al found that PGC-1 _amp_#x003b1 target genes (NDUFS3, NDUFS3 CYCS COX6A1 NDUFB5 ACADM TFAM | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | However they showed that PGC-1 _amp_#x003b1 expression is decreased in the striatum of human HD | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | PGC-1 _amp_#x003b1 has been implicated in mitochondrial biogenesis through its ability | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Activated SIRT1 leads to deactylation of PGC-1 _amp_#x003b1 resulting in an activation of PGC-1 _amp_#x003b1 73 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | to deactylation of PGC-1 _amp_#x003b1 resulting in an activation of PGC-1 _amp_#x003b1 73 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | By deacetylating PGC-1 _amp_#x003b1 SIRT1 represses glycolysis increase hepatic glucose output and modulates | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | PGC-1 _amp_#x003b1 is known as master regulator of mitochondrial biogenesis and | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Thiazolidiones and rexinoids induce PGC-1 _amp_#x003b1 gene transcription in brown and white adipocytes 75 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Based on the studies on PGC-1 _amp_#x003b1 knockout mice that shown to have neurodegenerative lesions particularly | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | However the neurodegenerative lesions in PGC-1 _amp_#x003b1 knockout mice do not mimic lesions in HD | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | PPAR-_amp_#x003b3 's natural coactivator is PGC-1 _amp_#x003b1 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | TZDs can mimic the effect of PGC-1 _amp_#x003b1 on PPAR-_amp_#x003b3 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | If PGC-1 _amp_#x003b1 levels reduces or become inactivated by acetylation then the | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | The activation of PGC-1 _amp_#x003b1 in HD mouse models or overexpression of PGC-1 _amp_#x003b1 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | Mitochondria have been implicated in ALS and PGC-1 _amp_#x003b1 has possible role in mitochondrial biogenesis therefore it would | | |
| 18464922 | PGC1 | PGC-1 | 1.2 | therefore it would be informative to examine mitochondrial abnormalities and PGC-1 _amp_#x003b1 in ALS | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | PGC1 | 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 | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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; . | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | impairment of pgc 1 _amp_#x003b1; could contribute to mitochondrial dysfunction in als. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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 | | |
| 18464922 | pgc 1 | 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 ]. | | |
| 18464922 | pgc 1 | 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 ]. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | however they showed that pgc 1 _amp_#x003b1; expression is decreased in the striatum of human hd. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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 | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | activated sirt1 leads to deactylation of pgc 1 _amp_#x003b1; resulting in an activation of pgc 1 _amp_#x003b1; [ 73 ]. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | by deacetylating pgc 1 _amp_#x003b1; sirt1 represses glycolysis increase hepatic glucose output and modulates mitochondrial function and biogenesis [ 73 ]. | | |
| 18464922 | pgc 1 | 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 | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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 ]. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | thiazolidiones and rexinoids induce pgc 1 _amp_#x003b1; gene transcription in brown and white adipocytes [ 75 ]. | | |
| 18464922 | pgc 1 | 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 ]. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | however the neurodegenerative lesions in pgc 1 _amp_#x003b1; knockout mice do not mimic lesions in hd. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | ppar _amp_#x003b3; 's natural coactivator is pgc 1 _amp_#x003b1; . | | |
| 18464922 | pgc 1 | pgc 1 | 1.0 | tzds can mimic the effect of pgc 1 _amp_#x003b1; on ppar _amp_#x003b3; . | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |
| 18464922 | pgc 1 | 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. | | |