Mar, 2011
Interaction of ataxin-3 with huntingtin-associated protein 1 through Josephin domain
NEUROREPORT
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- Volume
- 22
- Number
- 5
- First page
- 232
- Last page
- 238
- Language
- English
- Publishing type
- Research paper (scientific journal)
- DOI
- 10.1097/WNR.0b013e32834505f4
- Publisher
- LIPPINCOTT WILLIAMS & WILKINS
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. NeuroReport 22:232-238 (C) 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.
- Link information
- ID information
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- DOI : 10.1097/WNR.0b013e32834505f4
- ISSN : 0959-4965
- Pubmed ID : 21386698
- Web of Science ID : WOS:000288155900008