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Juvenile Huntington’s Disease Skin Fibroblasts Respond with Elevated Parkin Level and Increased Proteasome Activity as a Potential Mechanism to Counterbalance the Pathological Consequences of Mutant Huntingtin Protein

Huntington’s disease (HD) is an inherited neurodegenerative disorder, caused by an abnormal polyglutamine (polyQ) expansion in the huntingtin protein (Htt). Mitochondrial dysfunction and impairment of the ubiquitin-proteasome system (UPS) are hallmarks of HD neurons. The extraneural manifestations o...

詳細記述

保存先:
書誌詳細
出版年:Int J Mol Sci
主要な著者: Aladdin, Azzam, Király, Róbert, Boto, Pal, Regdon, Zsolt, Tar, Krisztina
フォーマット: Artigo
言語:Inglês
出版事項: MDPI 2019
主題:
オンライン・アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC6861992/
https://ncbi.nlm.nih.gov/pubmed/31717806
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms20215338
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