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Article

Leucine 7 is a key residue for mutant huntingtin-induced mitochondrial pathology and neurotoxicity in Huntington's disease.

The Journal of biological chemistry - 1 Mar 2025

Zhang Shengrong, Wang Shengda, Yang Zeyue, Li Yuanbo, Li Jinping, Chen Xushen, Yao Hao, Zheng Zhilong, Guo Xing

Abstract excerpt

Huntington's disease (HD) is a neurodegenerative disorder caused by the abnormal expansion of CAG repeats in exon 1 of the HTT gene. Mutant huntingtin (mHTT) associates with mitochondria, resulting in mitochondrial dysfunction and neuronal cell death. However, the underlying molecular mechanisms remain unknown. In this study, we investigate the role of N-terminal first 17 amino acids (N17) of mHTT in regulating...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 39947473
DOI: 10.1016/j.jbc.2025.108297
PMCID: PMC11930128

Genes

HTT

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

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Leucine 7 is a key residue for mutant huntingtin-induced mitochondrial pathology and neurotoxicity in Huntington's disease.PMID 39947473Read ↗

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