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Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.

Human molecular genetics - 1 Apr 2011

Havel Lauren S, Wang Chuan-En, Wade Brandy, Huang Brenda, Li Shihua, Li Xiao-Jiang

Abstract excerpt

An expanded polyglutamine tract (>37 glutamines) in the N-terminal region of huntingtin (htt) causes htt to accumulate in the nucleus, leading to transcriptional dysregulation in Huntington disease (HD). In HD knock-in mice that express full-length mutant htt at the endogenous level, mutant htt preferentially accumulates in the nuclei of striatal neurons, which are affected most profoundly in HD. The mechanism...

Topics

Animals
Corpus Striatum
HEK293 Cells
Humans
Huntingtin Protein
Huntington Disease
Mice
Mice, Mutant Strains
Mutation
Nerve Tissue Proteins
Neurons

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Nuclear Pore

Nuclear Proteins

Phosphorylation

Polyglutamic Acid

Protein Structure, Tertiary

Source

Open in PubMed Open full text

Identifiers

PMID: 21245084
DOI: 10.1093/hmg/ddr023
PMCID: PMC3049362

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

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Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation.PMID 21245084Read ↗

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