title: Instability of expressed Cu/Zn superoxide dismutase with 2 bp deletion found in familial amyotrophic lateral sclerosis.
abstract: The mutant Cu/Zn superoxide dismutase (SOD1) associated with familial amyotrophic lateral sclerosis (FALS) with a 2 bp deletion was produced in two protein expression systems. The mutant SOD1, expressed as a fusion protein in E. coli, had immunoreactivity to an anti-human SOD1 antibody but no SOD activity. It was more susceptible to proteolysis and its immunoreactivity decreased more rapidly than the wild type. The mutant SOD1, expressed in Cos1 cells, was not detected by either SOD activity staining or Western blot analysis, although expression of its mRNA was confirmed. These results suggest that the mutant SOD1 is seriously unstable in mammalian cells.
authors.affiliation: Division of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Nishimachi, Yonago, Japan. yawatana@grape.med.tottori-u.ac.jp
authors.forename: Y
authors.initials: Y
authors.lastname: Watanabe
authors.forename: Y
authors.initials: Y
authors.lastname: Kono
authors.forename: E
authors.initials: E
authors.lastname: Nanba
authors.forename: E
authors.initials: E
authors.lastname: Ohama
authors.forename: K
authors.initials: K
authors.lastname: Nakashima
mesh_terms: D018528:ATP-Binding Cassette Transporters; D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology* / Q000235:genetics; D000818:Animals; D019556:COS Cells; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism; D003300:Copper / Q000378:metabolism; D010450:Endopeptidases / Q000378:metabolism; D004795:Enzyme Stability / Q000235:genetics; D004926:Escherichia coli; D029968:Escherichia coli Proteins; D006801:Humans; D058619:Maltose-Binding Proteins; D051379:Mice; D008822:Mice, Transgenic; D009004:Monosaccharide Transport Proteins; D016297:Mutagenesis, Site-Directed; D011993:Recombinant Fusion Proteins / Q000235:genetics / Q000302:isolation & purification / Q000378:metabolism; D017384:Sequence Deletion; D013482:Superoxide Dismutase / Q000235:genetics / Q000302:isolation & purification / Q000378:metabolism*
chemical_list: D018528:ATP-Binding Cassette Transporters; D002352:Carrier Proteins; D029968:Escherichia coli Proteins; D058619:Maltose-Binding Proteins; D009004:Monosaccharide Transport Proteins; D011993:Recombinant Fusion Proteins; C461599:maltose transport system, E coli; D003300:Copper; D013482:Superoxide Dismutase; D010450:Endopeptidases