title: Transduced HSP27 protein protects neuronal cell death by enhancing FALS-associated SOD1 mutant activity.
abstract: Familial Amyotrophic lateral sclerosis (FALS) is a progressive neurodegenetative disorder induced by mutations of the SOD1 gene. Heat shock protein 27 (HSP27) is well-defined as a stress-inducible protein, however the its role in ALS protection has not yet been established. To investigate the role HSP27 may have in SOD1 mutant-mediated apoptosis, human SOD1 or HSP27 genes were fused with a PEP-1 peptide in a bacterial expression vector to produce a genetic in-frame fusion protein, which was then transduced into cells. We found the purified PEP-1-HSP27 fusion proteins can be transduced efficiently into neuronal cells and protect against cell death by enhancing mutant SOD1 activity. Moreover, transduced PEP-1-HSP27 efficiently prevents protein aggregation produced by oxidative stress. These results suggest that transduced HSP27 fusion protein may be explored as a potential therapeutic agent for FALS patients.
authors.affiliation: Department of Biomedical Science and Research Institute for Bioscience and Biotechnology, Hallym University, Chunchon 200-702, Korea.
authors.forename: Jae Jin
authors.initials: JJ
authors.lastname: An
authors.forename: Yeom Pyo
authors.initials: YP
authors.lastname: Lee
authors.forename: Dae Won
authors.initials: DW
authors.lastname: Kim
authors.forename: Eun Joung
authors.initials: EJ
authors.lastname: Sohn
authors.forename: Hoon Jae
authors.initials: HJ
authors.lastname: Jeong
authors.forename: Hye Won
authors.initials: HW
authors.lastname: Kang
authors.forename: Min Jae
authors.initials: MJ
authors.lastname: Shin
authors.forename: Mi Jin
authors.initials: MJ
authors.lastname: Kim
authors.forename: Eun Hee
authors.initials: EH
authors.lastname: Ahn
authors.forename: Sang Ho
authors.initials: SH
authors.lastname: Jang
authors.forename: Jung Hoon
authors.initials: JH
authors.lastname: Kang
authors.forename: Tae-Cheon
authors.initials: TC
authors.lastname: Kang
authors.forename: Moo Ho
authors.initials: MH
authors.lastname: Won
authors.forename: Oh-Shin
authors.initials: OS
authors.lastname: Kwon
authors.forename: Sung-Woo
authors.initials: SW
authors.lastname: Cho
authors.forename: Kil Soo
authors.initials: KS
authors.lastname: Lee
authors.forename: Jinseu
authors.initials: J
authors.lastname: Park
authors.forename: Won Sik
authors.initials: WS
authors.lastname: Eum
authors.forename: Soo Young
authors.initials: SY
authors.lastname: Choi