title: Assays for studying nucleated aggregation of polyglutamine proteins.
abstract: The aggregation of polyglutamine containing protein sequences is implicated in a family of familial neurodegenerative diseases, the expanded CAG repeat diseases. While the cellular aggregation process undoubtedly depends on the flux and local environment of these proteins, their intrinsic physical properties and folding/aggregation propensities must also contribute to their cellular behavior. Here we describe a series of methods for determining mechanistic details of the spontaneous aggregation of polyQ-containing sequences, including the identification and structural examination of aggregation intermediates.
authors.affiliation: Department of Structural Biology and Pittsburgh Institute for Neurodegenerative Diseases, Biomedical Sciences Tower 3, 3501 Fifth Avenue, Pittsburgh, PA 15260, USA.
authors.forename: Murali
authors.initials: M
authors.lastname: Jayaraman
authors.forename: Ashwani K
authors.initials: AK
authors.lastname: Thakur
authors.forename: Karunakar
authors.initials: K
authors.lastname: Kar
authors.forename: Ravindra
authors.initials: R
authors.lastname: Kodali
authors.forename: Ronald
authors.initials: R
authors.lastname: Wetzel
mesh_terms: D052160:Benzothiazoles; D005470:Fluorometry; D006801:Humans; D000071058:Huntingtin Protein; D015151:Immunoblotting; D007700:Kinetics; D008854:Microscopy, Electron; D046912:Multiprotein Complexes / Q000737:chemistry; D009419:Nerve Tissue Proteins / Q000737:chemistry*; D009687:Nuclear Proteins / Q000737:chemistry*; D010455:Peptides / Q000737:chemistry*; D055503:Protein Multimerization; D017434:Protein Structure, Tertiary; D013844:Thiazoles / Q000737:chemistry; D014461:Ultracentrifugation
chemical_list: D052160:Benzothiazoles; C086055:HTT protein, human; D000071058:Huntingtin Protein; D046912:Multiprotein Complexes; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D010455:Peptides; D013844:Thiazoles; C009462:thioflavin T; C097188:polyglutamine