Interaction of ataxin-3 with huntingtin-associated protein 1 through Josephin domain.
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.
Mesh Terms:
Blotting, Western, Humans, Immunoprecipitation, Nerve Tissue Proteins, Neurons, Nuclear Proteins, Protein Binding, Protein Structure, Tertiary, Repressor Proteins, Reverse Transcriptase Polymerase Chain Reaction, Spinocerebellar Ataxias, Transfection
Blotting, Western, Humans, Immunoprecipitation, Nerve Tissue Proteins, Neurons, Nuclear Proteins, Protein Binding, Protein Structure, Tertiary, Repressor Proteins, Reverse Transcriptase Polymerase Chain Reaction, Spinocerebellar Ataxias, Transfection
Neuroreport
Date: Mar. 30, 2011
PubMed ID: 21386698
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