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HAP40 modulates mutant Huntingtin aggregation and toxicity in Huntington's disease mice

Cell Death Dis. 2024-05; 
Laiqiang Chen, Yiyang Qin, Tingting Guo, Wenzhen Zhu, Jingpan Lin, Tingting Xing, Xuezhi Duan, Yiran Zhang, Eshu Ruan, Xiang Li, Peng Yin, Shihua Li, Xiao-Jiang Li, Su Yang
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Proteins, Expression, Isolation and Analysis … of mHTT aggregation in the mammalian brain and hinted a promising … protein in the samples was separated by SDS-PAGE electrophoresis using SurePAGE? Bis-Tris Gels (GenScript, … Get A Quote

摘要

Huntington's disease (HD) is a monogenic neurodegenerative disease, caused by the CAG trinucleotide repeat expansion in exon 1 of the Huntingtin (HTT) gene. The HTT gene encodes a large protein known to interact with many proteins. Huntingtin-associated protein 40 (HAP40) is one that shows high binding affinity with HTT and functions to maintain HTT conformation in vitro. However, the potential role of HAP40 in HD pathogenesis remains unknown. In this study, we found that the expression level of HAP40 is in parallel with HTT but inversely correlates with mutant HTT aggregates in mouse brains. Depletion of endogenous HAP40 in the striatum of HD140Q knock-in (KI) mice leads to enhanced mutant HTT aggregation and ... More

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