title: Small heat shock proteins in inherited peripheral neuropathies.
abstract: Small heat shock proteins (small HSPs) are molecular chaperones that protect cells against stress by assisting in the correct folding of denatured proteins and thus prevent aggregation of misfolded proteins. Small HSPs also modulate apoptotic pathways by interacting with components of programmed cell death. Furthermore, some small HSPs interact with the cytoskeleton to assist in spatial organization and dynamics of its structural elements. The role of small HSPs has been studied in many disorders, including neurodegenerative disease. Recently, mutations in HSPB1 (HSP27) and HSPB8 (HSP22), two members of the small HSP superfamily, have been associated with inherited peripheral neuropathies. In this review, we will summarize the current knowledge of small HSPs, in particular HSPB1 and HSPB8, and discuss their role in health and disease.
authors.affiliation: Department of Molecular Genetics, Flanders Interuniversity Institute for Biotechnology, Institute Born-Bunge, University of Antwerp, Antwerpen, Belgium.
authors.forename: Ines
authors.initials: I
authors.lastname: Dierick
authors.forename: Joy
authors.initials: J
authors.lastname: Irobi
authors.forename: Peter
authors.initials: P
authors.lastname: De Jonghe
authors.forename: Vincent
authors.initials: V
authors.lastname: Timmerman
mesh_terms: D050888:Heat-Shock Proteins, Small / Q000502:physiology*; D006801:Humans; D010523:Peripheral Nervous System Diseases / Q000821:virology*
chemical_list: D050888:Heat-Shock Proteins, Small