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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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Podrobná bibliografie
Vydáno v:Int J Mol Sci
Hlavní autoři: Aladdin, Azzam, Király, Róbert, Boto, Pal, Regdon, Zsolt, Tar, Krisztina
Médium: Artigo
Jazyk:Inglês
Vydáno: MDPI 2019
Témata:
On-line přístup: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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