title: Are expanded polyglutamine proteins a proteasome substrate?
abstract: Protein degradation is essential to preserve cellular homeostasis since it is an important step in a variety of cellular processes. Among the intracellular proteolytic systems, the proteasome is considered to play a major role in basal protein turnover, that is, degradation of abnormal/damaged proteins. The accumulation of protein aggregates as well as various components of the ubiquitin/proteasome system raises the possibility that an impaired proteasome function may be a causal factor in the cellular degeneration that occurs in neurodegenerative disorders caused by expanded polyglutamine repeats.
authors.affiliation: Laboratoire de Biologie et Biochimie Cellulaire du Vieillissement, Université Paris 7--Denis Diderot, Paris, France.
authors.forename: Anne-Laure
authors.initials: AL
authors.lastname: Bulteau
authors.forename: Bertrand
authors.initials: B
authors.lastname: Friguet
mesh_terms: D000818:Animals; D042622:DNA Repeat Expansion; D006801:Humans; D019636:Neurodegenerative Diseases / Q000235:genetics; D010455:Peptides / Q000235:genetics* / Q000378:metabolism*; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism*
chemical_list: D010455:Peptides; C097188:polyglutamine; D046988:Proteasome Endopeptidase Complex