Article
Identification of a misfolded region in superoxide dismutase 1 that is exposed in amyotrophic lateral sclerosis.
The Journal of biological chemistry - 10 Oct 2014
Rotunno Melissa S, Auclair Jared R, Maniatis Stephanie, Shaffer Scott A, Agar Jeffrey, Bosco Daryl A
Abstract excerpt
Mutations and aberrant post-translational modifications within Cu,Zn-superoxide dismutase (SOD1) cause this otherwise protective enzyme to misfold, leading to amyotrophic lateral sclerosis (ALS). The C4F6 antibody selectively binds misfolded SOD1 in spinal cord tissues from postmortem human ALS cases, as well as from an ALS-SOD1 mouse model, suggesting that the C4F6 epitope reports on a pathogenic conformation...
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