title: Inclusion formation by ataxins -1, -2, -3, and -7.
abstract: The authors studied inclusion formation in vitro using transiently transfected PC12 cells, with epitope-tagged and untagged full-length and truncated wild-type and expanded ataxins -1, -2, -3, and -7. At 72 hours, no inclusions were seen with wild-type full-length or truncated ataxins -2, -3, or -7, and only one with ataxin-1. Truncation abolished nuclear localization of ataxins -1 and -7, and allowed nuclear entry of ataxin-2. Of the expanded ataxins, only -1 and -2 formed inclusions, and those of ataxin-2 were rare and exclusively cytoplasmic. Truncation resulted in inclusion formation by ataxins -3 and -7, increased ataxin-1 inclusions, and enabled formation of nuclear ataxin-2 inclusions. There was no recruitment of wild-type ataxin-1 to expanded ataxin-1 inclusions.
authors.affiliation: Van Cleef Roet Centre for Nervous Diseases, Department of Medicine (Neuroscience), Monash University (Alfred Hospital Campus), Melbourne, VIC, Australia.
authors.forename: Volga
authors.initials: V
authors.lastname: Tarlac
authors.forename: Victor
authors.initials: V
authors.lastname: Turnbull
authors.forename: Daniela
authors.initials: D
authors.lastname: Stefani
authors.forename: Louise
authors.initials: L
authors.lastname: Kelly
authors.forename: Renae
authors.initials: R
authors.lastname: Walsh
authors.forename: Elsdon
authors.initials: E
authors.lastname: Storey
mesh_terms: D000818:Animals; D000067696:Ataxin-1; D000067699:Ataxin-3; D000067719:Ataxin-7; D000067528:Ataxins; D002479:Inclusion Bodies / Q000378:metabolism*; D009154:Mutation / Q000502:physiology; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000502:physiology*; D009687:Nuclear Proteins / Q000235:genetics / Q000502:physiology; D016716:PC12 Cells; D051381:Rats; D014162:Transfection / Q000379:methods
chemical_list: D000067696:Ataxin-1; D000067719:Ataxin-7; D000067528:Ataxins; C000593751:Atxn1 protein, rat; C000593832:Atxn7 protein, rat; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D000067699:Ataxin-3; C511593:Atxn3 protein, rat