HUGO ID Detailed Result 4851


HUGO ID 4851
Symbol HTT
Name huntingtin
#Occurrence 12
#Paper 3

 


PMID Match String Actual String Score Flanking text Edited by Edit
14739060huntingtinhuntingtin1.0the increased number in gag repeats in the mutant gene are expressed as an elongated huntingtin protein [ 38 ].  
15571972huntingtinhuntingtin1.0recent studies have reported that a daily treatment with minocycline 5_amp_#x2013;10 mg/kg i.p. slows progression and delays mortality of mice carrying the exon 1 of mutated huntingtin with 144 cag repeats when administrated from 8.5 weeks of age carreras et al. 2003 chen et al. 2000 wang et al. 2003b and wang et al. 2003a .  
15571972huntingtinhuntingtin1.0in vitro minocycline 5_amp_#x2013;100 _amp_#x3bc;m inhibits cell death and apoptosis induced by either mutated huntingtin or other neurotoxic stimulations promoting caspase activation blum et al. unpublished observations; wang et al. 2003a .  
15571972huntingtinhuntingtin1.0 2003 using a slice culture model of hd clearly demonstrated that 30 _amp_#x3bc;m minocycline inhibit huntingtin aggregation up to tenfold.  
15571972huntingtinhuntingtin1.0however this is inconsistent with other recent in vitro data showing that 50 _amp_#x3bc;m minocycline fails to inhibit aggregation in cells overexpressing mutated huntingtin apostol et al. 2003 .  
18464922huntingtinhuntingtin1.0like 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.  
18464922huntingtinhuntingtin1.0the 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.  
18464922huntingtinhuntingtin1.0the polyglutamine expansion causes the aggregation of huntingtin protein and formation of neuronal inclusion bodies as reviewed by ortega et al. [ 50 ].  
18464922huntingtinhuntingtin1.0ively 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.  
18464922huntingtinhuntingtin1.0recent 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 
18464922huntingtinhuntingtin1.0 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 ].  
18464922huntingtinhuntingtin1.0weydt 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 ].