The APCs of neuroprotection.
Mutations in the enzyme superoxide dismutase 1 (SOD1) have been linked to the neurodegenerative disease amyotrophic lateral sclerosis (ALS). In this issue of the JCI, Zhong et al. report that the endogenous anticoagulant activated protein C (APC) is able to cross the blood-spinal cord barrier in mice and signal to both neuronal and non-neuronal cells (see the related article beginning on page 3437). This signaling resulted in the suppression of mutant SOD1 synthesis and retarded disease progression in a murine model of ALS. Here we discuss the potential importance of these data and possible relevance to human neurodegenerative diseases.
Esmon
Charles T
CT
Cardiovascular Biology Research Program, Oklahoma Medical Research Foundation, and Howard Hughes Medical Institute, Oklahoma City, Oklahoma 73104, USA. Charles-Esmon@omrf.org
Glass
Jonathan D
JD
D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000503:physiopathology; D000818:Animals; D000925:Anticoagulants / Q000378:metabolism / Q000494:pharmacology / Q000627:therapeutic use; D001812:Blood-Brain Barrier / Q000378:metabolism; D004195:Disease Models, Animal; D015971:Gene Expression Regulation, Enzymologic / Q000187:drug effects; D006801:Humans; D051379:Mice; D019636:Neurodegenerative Diseases / Q000188:drug therapy / Q000503:physiopathology*; D011486:Protein C / Q000378:metabolism* / Q000494:pharmacology / Q000627:therapeutic use; D015398:Signal Transduction
D000925:Anticoagulants; D011486:Protein C