Ontology highlight
ABSTRACT:
SUBMITTER: Chattopadhyay M
PROVIDER: S-EPMC2585484 | biostudies-literature | 2008 Dec
REPOSITORIES: biostudies-literature
Chattopadhyay Madhuri M Durazo Armando A Sohn Se Hui SH Strong Cynthia D CD Gralla Edith B EB Whitelegge Julian P JP Valentine Joan Selverstone JS
Proceedings of the National Academy of Sciences of the United States of America 20081120 48
Familial amyotrophic lateral sclerosis (fALS) caused by mutations in copper-zinc superoxide dismutase (SOD1) is characterized by the presence of SOD1-rich inclusions in spinal cords. Similar inclusions observed in fALS transgenic mice have a fibrillar appearance suggestive of amyloid structure. Metal-free apo-SOD1 is a relatively stable protein and has been shown to form amyloid fibers in vitro only when it has been subjected to severely destabilizing conditions, such as low pH or reduction of i ...[more]