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Ubiquitin-activating enzyme activity contributes to differential accumulation of mutant huntingtin in brain and peripheral tissues.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 18 Jun 2014

Wade Brandy E, Wang Chuan-En, Yan Sen, Bhat Kavita, Huang Brenda, Li Shihua, Li Xiao-Jiang

Abstract excerpt

Huntington's disease (HD) belongs to a family of neurodegenerative diseases caused by misfolded proteins and shares the pathological hallmark of selective accumulation of misfolded proteins in neuronal cells. Polyglutamine expansion in the HD protein, huntingtin (Htt), causes selective neurodegeneration that is more severe in the striatum and cortex than in other brain regions, but the mechanism behind this...

Topics

Animals
Brain Chemistry
Enzyme Activation
Female
Gene Knock-In Techniques
HEK293 Cells
Humans
Huntingtin Protein
Male
Mice
Mutation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Nerve Tissue Proteins

Nuclear Proteins

Tissue Distribution

Ubiquitin-Activating Enzymes

Source

Open in PubMed Open full text

Identifiers

PMID: 24948797
DOI: 10.1523/JNEUROSCI.0775-14.2014
PMCID: PMC4061386

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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Ubiquitin-activating enzyme activity contributes to differential accumulation of mutant huntingtin in brain and peripheral tissues.PMID 24948797Read ↗

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