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Interaction of ataxin-3 with huntingtin-associated protein 1 through Josephin domain

Takeshita, Yukioa,b; Fujinaga, Ryutaroa; Kokubu, Keijia; Islam, Md. Nabiula; Jahan, Mir Rubayeta; Yanai, Akiea; Kakizuka, Akirac; Shinoda, Koha

doi: 10.1097/WNR.0b013e32834505f4
MOLECULAR NEUROSCIENCE
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Huntingtin-associated protein 1 (HAP1) is an essential component of the stigmoid body (STB) and known as a possible neuroprotective interactor with causative proteins for Huntington's disease, spinal and bulbar muscular atrophy, spinocerebellar ataxia type 17 (SCA17), and Joubert syndrome. To clarify what other causative molecules HAP1/STB could interact with, we cloned normal causative genes for several neural disorders from human brain RNA library and evaluated their subcellular interaction with HAP1/STB by immunocytochemistry and immunoprecipitation after cotransfection into Neuro2a cells. The results clearly showed that HAP1/STB interacts with the normal ataxin-3 through Josephin domain and polyglutamine-expanded mutants derived from SCA3 as well. The findings suggest that HAP1/STB could modify the physiological function of normal ataxin-3 and pathogenesis of SCA3 attributable to the mutant ataxin-3.

Supplemental Digital Content is available in the text.

aDepartment of Neuroscience, Yamaguchi Graduate University School of Medicine, Ube, Yamaguchi, Japan

bThe Japan Society for the Promotion of science, Tokyo

cLaboratory of Functional Biology, Graduate School of Biostudies, Kyoto University, Kyoto, Japan

Correspondence to Dr Koh Shinoda, Department of Neuroscience, Yamaguchi University School of Medicine, 1-1-1 Minami-Kogushi, Ube, Yamaguchi 755-8505, Japan Tel: +81 836 22 2204; fax: +81 836 22 2205; e-mail: shinoda@yamaguchi-u.ac.jp

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.neuroreport.com).

Received December 14, 2010

Accepted January 24, 2011

© 2011 Lippincott Williams & Wilkins, Inc.