VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington’s disease

Xing Guo; XiaoYan Sun; Di Hu; Ya-Juan Wang; Hisashi Fujioka; Rajan Vyas; Sudha Chakrapani; Amit Umesh Joshi; Yu Luo; Daria Mochly-Rosen; Xin Qi

doi:10.1038/ncomms12646

Nature Communications (Aug 2016)

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington’s disease

Xing Guo,
XiaoYan Sun,
Di Hu,
Ya-Juan Wang,
Hisashi Fujioka,
Rajan Vyas,
Sudha Chakrapani,
Amit Umesh Joshi,
Yu Luo,
Daria Mochly-Rosen,
Xin Qi

Affiliations

Xing Guo: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine
XiaoYan Sun: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine
Di Hu: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine
Ya-Juan Wang: Center for Proteomics and Bioinformatics, Case Western Reserve University School of Medicine
Hisashi Fujioka: Department of Pharmacology, Case Western Reserve University School of Medicine
Rajan Vyas: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine
Sudha Chakrapani: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine
Amit Umesh Joshi: Department of Chemical and Systems Biology, Stanford University School of Medicine
Yu Luo: Department of Neurosurgery, Case Western Reserve University School of Medicine
Daria Mochly-Rosen: Department of Chemical and Systems Biology, Stanford University School of Medicine
Xin Qi: Department of Physiology & Biophysics, Case Western Reserve University School of Medicine

DOI: https://doi.org/10.1038/ncomms12646
Journal volume & issue: Vol. 7, no. 1
pp. 1 – 17

Abstract

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Mitochondria defects caused by mutant huntingtin (mtHtt) have been implicated in Huntington's disease. Here authors show that VCP binds to mtHtt on the mitochondria, and that treatment with a peptide that disrupts this interaction reduces the cellular and behavioural deficits in mouse models of HD.

Published in Nature Communications

ISSN: 2041-1723 (Online)
Publisher: Nature Portfolio
Country of publisher: United Kingdom
LCC subjects: Science
Website: https://www.nature.com/ncomms/

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