title: Heat-shock proteins in clinical neurology.
abstract: Heat-shock proteins (HSPs) are antigen-presenting protein-aggregation-preventing chaperones, induced by cellular stress in eukaryotic cells. In this review, we focus on recent HSP advances in neurological disorders. In myasthenia gravis, patients responding to immunosuppressive therapy have reduced serum HSP-71 antibodies. Generalized and ocular myasthenia gravis patients have elevated serum HSP-70 antibodies, indicating common pathogenic mechanisms. In Guillain-Barré syndrome, HSP-70 antibodies are elevated in serum and cerebrospinal fluid, and serum levels are higher than in myasthenia gravis and multiple sclerosis. In multiple sclerosis, serum HSP-27 antibodies are elevated during relapses providing disease activation marker, while α,β-crystallin expression in brain lesions indicates remission phase initiation. In acute stroke, serum HSP-27 antibodies are elevated irrespective of stroke type and duration. In epilepsy, HSP-27 is induced in patients' astrocytes and cerebral blood vessel walls, and α,β-crystallin is expressed in epileptic foci. In neurodegenerative disorders such as Alzheimer dementia and Parkinson's disease, HSPs are upregulated in brain tissue, and α,β-crystallin modulates superoxide dismutase-1 (SOD-1) tissue accumulation in familial amyotrophic lateral sclerosis. HSPs play an important role in antigen-presentation and tolerance development. Antibody-mediated interference with their function alters immune responses causing neuropathology. The role of HSPs in clinical neurology should be the subject of future investigation.
authors.affiliation: Department of Neurology, Haukeland University Hospital, Bergen, Norway. fredrik.romi@haukeland.no
authors.forename: Fredrik
authors.initials: F
authors.lastname: Romi
authors.forename: Geir
authors.initials: G
authors.lastname: Helgeland
authors.forename: Nils Erik
authors.initials: NE
authors.lastname: Gilhus
mesh_terms: D006360:Heat-Shock Proteins / Q000378:metabolism*; D006801:Humans; D009422:Nervous System Diseases / Q000145:classification / Q000378:metabolism* / Q000473:pathology; D009462:Neurology
chemical_list: D006360:Heat-Shock Proteins