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Article

Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.

The Journal of cell biology - 13 Oct 2003

Zhou Hui, Cao Fengli, Wang Zhishan, Yu Zhao-Xue, Nguyen Huu-Phuc, Evans Joy, Li Shi-Hua, Li Xiao-Jiang

Abstract excerpt

Although NH2-terminal mutant huntingtin (htt) fragments cause neurological disorders in Huntington's disease (HD), it is unclear how toxic htt fragments are generated and contribute to the disease process. Here, we report that complex NH2-terminal mutant htt fragments smaller than the first 508 amino acids were generated in htt-transfected cells and HD knockin mouse brains. These fragments constituted neuronal...

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Identifiers

PMID: 14557250
DOI: 10.1083/jcb.200306038
PMCID: PMC2173440

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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