Ontology highlight
ABSTRACT:
SUBMITTER: Allen MJ
PROVIDER: S-EPMC3384721 | biostudies-literature | 2012 Mar
REPOSITORIES: biostudies-literature
Allen Michael J MJ Lacroix Jérome J JJ Ramachandran Srinivasan S Capone Ricardo R Whitlock Jenny L JL Ghadge Ghanashyam D GD Arnsdorf Morton F MF Roos Raymond P RP Lal Ratnesh R
Neurobiology of disease 20110910 3
Point mutations in the gene encoding copper-zinc superoxide dismutase (SOD1) impart a gain-of-function to this protein that underlies 20-25% of all familial amyotrophic lateral sclerosis (FALS) cases. However, the specific mechanism of mutant SOD1 toxicity has remained elusive. Using the complementary techniques of atomic force microscopy (AFM), electrophysiology, and cell and molecular biology, here we examine the structure and activity of A4VSOD1, a mutant SOD1. AFM of A4VSOD1 reconstituted in ...[more]