title: The emerging family of dystrophin-related proteins.
abstract: Duchenne and Becker muscular dystrophies are caused by mutations in the gene encoding dystrophin, a component of the subsarcolemmal cytoskeleton. Dystrophin-related proteins are identical or homologous to the cysteine-rich and C-terminal domains of dystrophin. This part of dystrophin binds to a membrane-spanning glycoprotein complex in muscle. At least five dystrophin-related proteins are encoded by the Duchenne muscular dystrophy locus. These proteins are found in many non-muscle tissues where dystrophin is not expressed and they are thought to be membrane-associated. Two other dystrophin-related proteins--utrophin and an 87 kDa postsynaptic protein--are encoded by separate loci and, like dystrophin, they are components of the neuromuscular junction.
authors.affiliation: Molecular Genetics Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, UK OX3 9DU.
authors.forename: D J
authors.initials: DJ
authors.lastname: Blake
authors.forename: J M
authors.initials: JM
authors.lastname: Tinsley
authors.forename: K E
authors.initials: KE
authors.lastname: Davies