Identification of alpha-syntrophin binding to syntrophin triplet, dystrophin, and utrophin.
Syntrophin represents three cytoplasmic components of the dystrophin-glycoprotein complex that links the cytoskeleton to the extracellular matrix in skeletal muscle. alpha-Syntrophin has now been translated in vitro and shown to associate directly with all three components of the syntrophin triplet and with dystrophin. The in vitro translated 71-kDa non-muscle dystrophin ... isoform, containing the cystein-rich/C-terminal domain, can also interact with the syntrophin triplet. The syntrophin binding motif in dystrophin was localized to exons 73 and 74 including amino acids 3447-3481 by comparing the interactions of alpha-syntrophin and seven overlapping human dystrophin fusion proteins. More than one syntrophin interaction site in this binding motif was suggested. alpha-Syntrophin also interacts directly with a C-terminal utrophin fusion protein. alpha-Syntrophin is localized to the muscle sarcolemma as well as to the neuromuscular junction in control mouse muscle. However, similar to utrophin, alpha-syntrophin is only present at the neuromuscular junction in mdx mouse muscle in which dystrophin is absent. Our data suggest that alpha-syntrophin binds all syntrophin isoforms, and syntrophin directly interacts with dystrophin through more than one binding site in dystrophin exons 73 and 74 including amino acids 3447-3481.
Mesh Terms:
ATP-Binding Cassette Transporters, Amino Acid Sequence, Animals, Bacterial Proteins, Base Sequence, Binding Sites, Calcium-Binding Proteins, Carrier Proteins, Cytoskeletal Proteins, DNA Primers, Dystrophin, Escherichia coli, Escherichia coli Proteins, Exons, Glutathione Transferase, Humans, Membrane Proteins, Molecular Sequence Data, Monosaccharide Transport Proteins, Muscle Proteins, Muscles, Polymerase Chain Reaction, Protein Biosynthesis, Rabbits, Recombinant Fusion Proteins, Transcription, Genetic, Utrophin
ATP-Binding Cassette Transporters, Amino Acid Sequence, Animals, Bacterial Proteins, Base Sequence, Binding Sites, Calcium-Binding Proteins, Carrier Proteins, Cytoskeletal Proteins, DNA Primers, Dystrophin, Escherichia coli, Escherichia coli Proteins, Exons, Glutathione Transferase, Humans, Membrane Proteins, Molecular Sequence Data, Monosaccharide Transport Proteins, Muscle Proteins, Muscles, Polymerase Chain Reaction, Protein Biosynthesis, Rabbits, Recombinant Fusion Proteins, Transcription, Genetic, Utrophin
J. Biol. Chem.
Date: Mar. 10, 1995
PubMed ID: 7890602
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