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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...

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Dettagli Bibliografici
Pubblicato in:Int J Mol Sci
Autori principali: Aladdin, Azzam, Király, Róbert, Boto, Pal, Regdon, Zsolt, Tar, Krisztina
Natura: Artigo
Lingua:Inglês
Pubblicazione: MDPI 2019
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Accesso online: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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