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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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Dades bibliogràfiques
Publicat a:Int J Mol Sci
Autors principals: Aladdin, Azzam, Király, Róbert, Boto, Pal, Regdon, Zsolt, Tar, Krisztina
Format: Artigo
Idioma:Inglês
Publicat: MDPI 2019
Matèries:
Accés en línia: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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