Regulation of Torsin ATPases by LAP1 and LULL1

TorsinA is a membrane-associated AAA+ (ATPases associated with a variety of cellular activities) ATPase implicated in primary dystonia, an autosomal-dominant movement disorder. We reconstituted TorsinA and its cofactors in vitro and show that TorsinA does not display ATPase activity in isolation; AT...

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Veröffentlicht in:	Proceedings of the National Academy of Sciences - PNAS 2013-04, Vol.110 (17), p.E1545-E1554
Hauptverfasser:	Zhao, Chenguang, Brown, Rebecca S H, Chase, Anna R, Eisele, Markus R, Schlieker, Christian
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenosine triphosphatase Adenosine Triphosphatases - metabolism adenosine triphosphate adenosinetriphosphatase Biological Sciences Carrier Proteins - metabolism Chromatography, Gel Cloning, Molecular Congenital diseases Dystonia Musculorum Deformans - genetics Dystonia Musculorum Deformans - metabolism endoplasmic reticulum Endoplasmic Reticulum - metabolism HEK293 Cells HeLa Cells HSC70 Heat-Shock Proteins - metabolism Humans Hydrolysis Immunoblotting Immunoprecipitation Membrane Proteins - metabolism Membranes Molecular Chaperones - genetics Molecular Chaperones - metabolism mutants Mutation Neurological disorders nuclear membrane PNAS Plus Proteins transmembrane proteins
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