title: Differential expression of small heat shock protein 27 (Hsp27) in Ataxia telangiectasia brains.
abstract: Ataxia telangiectasia (A-T) is a progressive neurodegenerative disorder caused by disruption of the gene, ataxia telangiectasia mutated (ATM). Present study was aimed at identifying proteins that are present in abnormal levels in A-T brain that may identify alternative targets for therapeutic interventions. Proteomic and Western blot analysis have shown massive expression of the small heat shock protein 27 (Hsp27) in frontal cortices of A-T brains compared to negligible levels in controls. The expression of other stress proteins, Hsp70, alphaB-crystallin, and prohibitin remained unchanged in the A-T and control brains. Significant decreases in reactive oxygen species, protein carbonyl groups and lipid peroxidation products were observed in the A-T brains. There is no evidence of caspase 3 activation or DAXX mediated apoptosis. We propose that neurons in the frontal lobe are protected by the expression of Hsp27, which scavenges the oxidative stress molecules formed consequent to the primary loss of ATM function.
authors.affiliation: Department of Developmental Biochemistry, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA.
authors.forename: Wenqiang
authors.initials: W
authors.lastname: Chen
authors.forename: Salomon
authors.initials: S
authors.lastname: Kuizon
authors.forename: Bair L
authors.initials: BL
authors.lastname: Chiou
authors.forename: David C
authors.initials: DC
authors.lastname: Bolton
authors.forename: Raju K
authors.initials: RK
authors.lastname: Pullarkat
authors.forename: Mohammed A
authors.initials: MA
authors.lastname: Junaid