Are expanded polyglutamine proteins a proteasome substrate?
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.
Bulteau
Anne-Laure
AL
Laboratoire de Biologie et Biochimie Cellulaire du Vieillissement, Université Paris 7--Denis Diderot, Paris, France.
Friguet
Bertrand
B
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*
D010455:Peptides; C097188:polyglutamine; D046988:Proteasome Endopeptidase Complex