Truncated Peroxisome Proliferator-Activated Receptor-γ Coactivator 1α Splice Variant Is Severely Altered in Huntington’s Disease | Zendy

Ashu Johri | Zendy; Anatoly A. Starkov | Zendy; Abhishek Chandra | Zendy; Thomas Hennessey | Zendy; Abhijeet Sharma | Zendy; S. Orobello | Zendy; Ferdinando Squitieri | Zendy; Lichuan Yang | Zendy; M. Flint Beal | Zendy

Open Access

Truncated Peroxisome Proliferator-Activated Receptor-γ Coactivator 1α Splice Variant Is Severely Altered in Huntington’s Disease

Author(s) -

Ashu Johri,

Anatoly A. Starkov,

Abhishek Chandra,

Thomas Hennessey,

Abhijeet Sharma,

S. Orobello,

Ferdinando Squitieri,

Lichuan Yang,

M. Flint Beal

Publication year - 2011

Publication title -

neurodegenerative diseases

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.98

H-Index - 57

eISSN - 1660-2862

pISSN - 1660-2854

DOI - 10.1159/000327910

Subject(s) - coactivator , mitochondrial biogenesis , biology , downregulation and upregulation , gene expression , ppargc1a , transgene , mitochondrion , genetically modified mouse , microbiology and biotechnology , gene , transcription factor , genetics

Reduced peroxisome proliferator-activated receptor-γ coactivator 1α (PGC1α) gene expression has been observed in striatal cell lines, transgenic mouse models of Huntington's disease (HD), and brain tissue from HD patients. As this protein is a key transcription regulator of the expression of many mitochondrial proteins, these observations strongly support the role of aberrant mitochondrial function in the pathogenesis of HD. The PGC1α protein undergoes posttranslational modifications that affect its transcriptional activity. The N-truncated splice variant of PGC1α (NT-PGC1α) is produced in tissues, but the role of truncated splice variants of PGC1α in HD and in the regulation of mitochondrial gene expression has not been elucidated.

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