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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Detalles Bibliográficos
Autores principales: Aladdin, Azzam, Király, Róbert, Boto, Pal, Regdon, Zsolt, Tar, Krisztina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6861992/
https://www.ncbi.nlm.nih.gov/pubmed/31717806
http://dx.doi.org/10.3390/ijms20215338

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6861992/
https://www.ncbi.nlm.nih.gov/pubmed/31717806
http://dx.doi.org/10.3390/ijms20215338

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