Mutant huntingtin and mitochondrial dysfunction
نویسندگان
چکیده
منابع مشابه
Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered energy metabolism have been implicated in HD pathogenesis. We report here that mutant huntingtin causes disruption of mitochondrial function by inhibiting expression of PGC-1alpha, a transcriptional coactivator that regulates ...
متن کاملP8: Temporal Lobe Epilepsy and Mitochondrial Dysfunction
لطفاً به چکیده انگلیسی مراجعه شود.
متن کاملhuntington’s disease and mitochondrial dna deletions: an incident or a regular mechanism for mutant huntingtin protein and cag repeats expansion?!
the mitochondrial dna (mtdna) may play an essential role in the pathogenesis of the respiratory chain complex activities in neurodegenerative disorders such as huntington’s disease (hd). research studies have been conducted to determine the possible levels of mitochondrial defect (deletion) in hd patients and the interaction between the expanded huntingtin gene as a nuclear gene and mitochondri...
متن کاملN-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.
Huntington's disease (HD) is caused by polyglutamine (polyQ) expansion in huntingtin (htt), a large (350 kDa) protein that localizes predominantly to the cytoplasm. Proteolytic cleavage of mutant htt yields polyQ-containing N-terminal fragments that are prone to misfolding and aggregation. Disease progression in HD transgenic models correlates with age-related accumulation of soluble and aggreg...
متن کاملRelationship between Mitochondrial Dysfunction and Multiple Sclerosis: A Review Study
Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system that inflammation, demyelination, oligodendrocyte loss, gliosis, axonal injury and neurodegeneration are the main histopathological hallmarks of the disease. Although MS was classically thought as a demyelinating disease, but axonal injury occurs commonly in acute inflammatory lesions. In MS mi...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Trends in Neurosciences
سال: 2008
ISSN: 0166-2236
DOI: 10.1016/j.tins.2008.09.004