Ontology highlight
ABSTRACT:
SUBMITTER: Lin AY
PROVIDER: S-EPMC3150896 | biostudies-literature | 2011 Aug
REPOSITORIES: biostudies-literature
Lin Ava Y AY Prochniewicz Ewa E James Zachary M ZM Svensson Bengt B Thomas David D DD
Proceedings of the National Academy of Sciences of the United States of America 20110718 31
We have used site-directed spin labeling and pulsed electron paramagnetic resonance to resolve a controversy concerning the structure of the utrophin-actin complex, with implications for the pathophysiology of muscular dystrophy. Utrophin is a homolog of dystrophin, the defective protein in Duchenne and Becker muscular dystrophies, and therapeutic utrophin derivatives are currently being developed. Both proteins have a pair of N-terminal calponin homology (CH) domains that are important for acti ...[more]