title: The BAG2 and BAG5 proteins inhibit the ubiquitination of pathogenic ataxin3-80Q.
abstract: The expansion of a polyglutamine domain in the protein ataxin3 causes spinocerebellar ataxia type-3 (SCA3). However, there is little information to date about the upstream proteins in the ubiquitin-proteasome system of pathogenic ataxin3-80Q. Here, we report that BAG2 (Bcl-2 associated athanogene family protein 2) and BAG5 (Bcl-2-associated athanogene family protein 5) stabilise pathogenic ataxin3-80Q by inhibiting its ubiquitination as determined based on western blotting and co-immunofluorescence experiments. The association of the BAG2 and BAG5 proteins with pathogenic ataxin3-80Q strengthens the important roles of the BAG family in neurodegenerative diseases.
authors.affiliation: 1The Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, People's Republic of China.
authors.forename: Xiang-Qian
authors.initials: XQ
authors.lastname: Che
authors.forename: Bei-Sha
authors.initials: BS
authors.lastname: Tang
authors.forename: Hong-Feng
authors.initials: HF
authors.lastname: Wang
authors.forename: Xin-Xiang
authors.initials: XX
authors.lastname: Yan
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
authors.forename: Lu
authors.initials: L
authors.lastname: Shen
authors.forename: Qian
authors.initials: Q
authors.lastname: Xu
authors.forename: Guang-Hui
authors.initials: GH
authors.lastname: Wang
authors.forename: Hai-Nan
authors.initials: HN
authors.lastname: Zhang
authors.forename: Chun-Yu
authors.initials: CY
authors.lastname: Wang
authors.forename: Ji-Feng
authors.initials: JF
authors.lastname: Guo
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics / Q000378:metabolism*; D000704:Analysis of Variance; D000067699:Ataxin-3 / Q000235:genetics / Q000378:metabolism*; D005786:Gene Expression Regulation / Q000235:genetics; D049452:Green Fluorescent Proteins / Q000235:genetics / Q000378:metabolism; D057809:HEK293 Cells; D006801:Humans; D047468:Immunoprecipitation; D018832:Molecular Chaperones / Q000235:genetics / Q000378:metabolism*; D010455:Peptides / Q000235:genetics / Q000378:metabolism*; D012097:Repressor Proteins / Q000235:genetics / Q000378:metabolism*; D014162:Transfection; D054875:Ubiquitination / Q000502:physiology*
chemical_list: D048868:Adaptor Proteins, Signal Transducing; C492980:BAG2 protein, human; C496515:BAG5 protein, human; D018832:Molecular Chaperones; D010455:Peptides; D012097:Repressor Proteins; C485184:enhanced green fluorescent protein; D049452:Green Fluorescent Proteins; C097188:polyglutamine; C092341:ATXN3 protein, human; D000067699:Ataxin-3
keywords: BAG2; BAG5; pathogenic ataxin3-80Q; spinocerebellar ataxia type-3; ubiquitin-proteasome system