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J Biol Chem, Vol. 273, Issue 37, 23625-23628, September 11, 1998
From the Edward Mallinckrodt Department of Pediatrics, Washington
University School of Medicine, St. Louis, Missouri 63110
Dominantly inherited mutations in the gene
encoding copper/zinc superoxide dismutase (SOD1) result in the fatal
motor neuron disease familial amyotrophic lateral sclerosis (FALS).
These mutations confer a gain-of-function to SOD1 with neuronal
degeneration resulting from enhanced free radical generating activity
of the copper present in the mutant enzyme. The delivery of copper to
SOD1 is mediated through a soluble factor identified as the copper
chaperone for SOD1 (CCS). Amino acid sequence alignment of SOD1 and CCS
reveals a striking homology with conservation of the amino acids
essential for mediating SOD1 homodimerization. Here we demonstrate that CCS and SOD1 directly interact in vitro and in
vivo and that this interaction is mediated via the homologous
domains in each protein. Importantly, CCS interacts not only with
wild-type SOD1 but also with SOD1 containing the common missense
mutations resulting in FALS. Our findings therefore reveal a common
mechanism whereby different SOD1 FALS mutants may result in neuronal
injury and suggest a novel therapeutic approach in patients affected by
this fatal disease.
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