[HTML][HTML] Transcriptional repression of PGC-1α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration

L Cui, H Jeong, F Borovecki, CN Parkhurst, N Tanese… - Cell, 2006 - cell.com
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a
glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered …

[HTML][HTML] Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1α in Huntington's disease neurodegeneration

P Weydt, VV Pineda, AE Torrence, RT Libby… - Cell metabolism, 2006 - cell.com
Huntington's disease (HD) is a fatal, dominantly inherited disorder caused by polyglutamine
repeat expansion in the huntingtin (htt) gene. Here, we observe that HD mice develop …

PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function

T Tsunemi, TD Ashe, BE Morrison… - Science translational …, 2012 - science.org
Huntington's disease (HD) is caused by CAG repeat expansions in the (huntingtin htt) gene,
yielding proteins containing polyglutamine repeats that become misfolded and resist …

[HTML][HTML] PGC-1α, a new therapeutic target in Huntington's disease?

JK McGill, MF Beal - Cell, 2006 - cell.com
The coactivator PGC-1α is a key regulator of mitochondrial biogenesis and respiration,
mediating expression of several transcription factors required for these programs. Three new …

The role of PGC-1α in the pathogenesis of neurodegenerative disorders

K Rona-Voros, P Weydt - Current drug targets, 2010 - ingentaconnect.com
Mitochondrial dysfunction is a common hallmark of ageing-related diseases involving
neurodegeneration. Huntington's disease (HD) is one of the most common monogenetic …

[HTML][HTML] Mitochondria in Huntington's disease

M Damiano, L Galvan, N Déglon, E Brouillet - Biochimica et Biophysica …, 2010 - Elsevier
Huntington's disease (HD) is an inherited progressive neurodegenerative disorder
associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric …

Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy …

RK Chaturvedi, NY Calingasan, L Yang… - Human molecular …, 2010 - academic.oup.com
We investigated the ability of AMP-activated protein kinase (AMPK) to activate PPARγ
coactivator-1α (PGC-1α) in the brain, liver and brown adipose tissue (BAT) of the NLS-N171 …

Expression profiling of Huntington's disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration

AD Strand, ZC Baquet, AK Aragaki… - Journal of …, 2007 - Soc Neuroscience
Many pathways have been proposed as contributing to Huntington's disease (HD)
pathogenesis, but generally the in vivo effects of their perturbation have not been compared …

PPARgamma rescue of the mitochondrial dysfunction in Huntington's disease

MC Chiang, Y Chern, RN Huang - Neurobiology of disease, 2012 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by
a CAG trinucleotide expansion in the Huntingtin (Htt) gene. The resultant mutant Htt protein …

Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease

RA Quintanilla, GVW Johnson - Brain research bulletin, 2009 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that is
caused by a pathological expansion of CAG repeats within the gene encoding for a 350kD …