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Article

Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo.

The Journal of biological chemistry - 31 Jul 2015

O'Brien Robert, DeGiacomo Francesco, Holcomb Jennifer, Bonner Akilah, Ring Karen L, Zhang Ningzhe, Zafar Khan, Weiss Andreas, Lager Brenda, Schilling Birgit, Gibson Bradford W, Chen Sylvia, Kwak Seung, Ellerby Lisa M

Abstract excerpt

The cascade of events that lead to cognitive decline, motor deficits, and psychiatric symptoms in patients with Huntington disease (HD) is triggered by a polyglutamine expansion in the N-terminal region of the huntingtin (HTT) protein. A significant mechanism in HD is the generation of mutant HTT fragments, which are generally more toxic than the full-length HTT. The protein fragments observed in human HD tissue...

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Identifiers

PMID: 26025364
DOI: 10.1074/jbc.M114.623561
PMCID: PMC4521048

Genes

HTT

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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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