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Jacob Ayers, Herman Lelie, Aron Workman, Mercedes Prudencio, Hilda Brown, Susan Fromholt, Joan Valentine, Julian Whitelegge and David Borchelt Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease Journal of Neurochemistry 128

Article first published online: 20 OCT 2013 | DOI: 10.1111/jnc.12451

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We sought to better characterize the biochemical features of two SOD1 mutants associated with rapidly progressing disease, the D101G and wild-type like D101N mutants. We observed using our cell model that that although similarities were observed when comparing the ability to bind metals and resist trypsin digestion, these mutants differed in their ability to initiate aggregation and to form the normal intramolecular disulfide bond. We conclude that these mutants exhibit distinct properties despite producing similar disease phenotypes in patients.

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