title: The small heat shock protein HspB8: role in nervous system physiology and pathology.
abstract: The accumulation and aggregation of misfolded proteins can be highly cytotoxic and may underlie several human degenerative diseases characterized by neuronal inclusions such as Alzheimer's, Parkinson's, prion-like and polyglutamine repeat diseases. In this context small heat shock proteins, molecular chaperones known to be induced by cell stress, play a fundamental role by facilitating folding of nascent polypeptides, preventing aggregation of misfolded proteins and enhancing their degradation. A recently identified member of the small heat shock protein family, HspB8, is of particular interest in the field of neurological diseases since mutations in its sequence correlate with development of distal hereditary motor neuropathy and Charcot-Marie-Tooth disease. HspB8 expression has been detected in neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, Huntington disease and spinocerebellar ataxia type 3. In the latter, HspB8 appears to be involved in protecting the cell from accumulation of insoluble aggregates either by preventing aggregation or by promoting degradation of improperly folded proteins. These data propose that HspB8 may be a major player in the neuroprotective response and a promising target for the development of therapeutic strategies.
authors.forename: Mattia
authors.initials: M
authors.lastname: Vicario
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Skaper
authors.affiliation: Department of Biomedical Sciences, University of Padua, Via G. Colombo 3, Padua 35121, Italy. alessandro.negro@unipd.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Negro
mesh_terms: D000818:Animals; D002319:Cardiovascular System / Q000378:metabolism; D006360:Heat-Shock Proteins / Q000378:metabolism*; D006801:Humans; D018832:Molecular Chaperones; D009420:Nervous System / Q000378:metabolism*; D009422:Nervous System Diseases / Q000473:pathology*; D017346:Protein Serine-Threonine Kinases / Q000378:metabolism*
chemical_list: C413214:HSPB8 protein, human; D006360:Heat-Shock Proteins; D018832:Molecular Chaperones; D017346:Protein Serine-Threonine Kinases