Disease-associated Mutations at Copper Ligand Histidine Residues of Superoxide Dismutase 1 Diminish the Binding of Copper and Compromise Dimer Stability

A subset of superoxide dismutase 1 (Cu/Zn-SOD1) mutants that cause familial amyotrophic lateral sclerosis (FALS) have heightened reactivity with -ONOO and H2O2in vitro. This reactivity requires a copper ion bound in the active site and is a suggested mechanism of motor neuron injury. However, we hav...

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Veröffentlicht in:	The Journal of biological chemistry 2007-01, Vol.282 (1), p.345-352
Hauptverfasser:	Wang, Jiou, Caruano-Yzermans, Amy, Rodriguez, Angela, Scheurmann, Jonathan P., Slunt, Hilda H., Cao, Xiaohang, Gitlin, Jonathan, Hart, P. John, Borchelt, David R.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Copper - chemistry Dimerization Histidine - chemistry Humans Ligands Mice Mice, Transgenic Models, Molecular Mutation Neurons - metabolism Protein Binding Superoxide Dismutase - chemistry Two-Hybrid System Techniques
Online-Zugang:	Volltext
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