title: The 2.0 A structure of the second calponin homology domain from the actin-binding region of the dystrophin homologue utrophin.
abstract: Utrophin is a close homologue of dystrophin, the protein defective in Duchenne muscular dystrophy. Like dystrophin, it is composed of three regions: an N-terminal region that binds actin filaments, a large central region with triple coiled-coil repeats, and a C-terminal region that interacts with components in the dystroglycan protein complex at the plasma membrane. The N-terminal actin-binding region consists of two calponin homology domains and is related to the actin-binding domains of a superfamily of proteins including alpha-actinin, spectrin and fimbrin. Here, we present the 2.0 A structure of the second calponin homology domain of utrophin solved by X-ray crystallography, and compare it to the other calponin homology domains previously determined from spectrin and fimbrin.
authors.affiliation: MRC Laboratory of Molecular Biology, Hills Road, Cambridge, CB2 2QH, UK. n.keep@mail.cryst.bbk.ac.uk
authors.forename: N H
authors.initials: NH
authors.lastname: Keep
authors.forename: F L
authors.initials: FL
authors.lastname: Norwood
authors.forename: C A
authors.initials: CA
authors.lastname: Moores
authors.forename: S J
authors.initials: SJ
authors.lastname: Winder
authors.forename: J
authors.initials: J
authors.lastname: Kendrick-Jones
mesh_terms: D000199:Actins / Q000378:metabolism; D000595:Amino Acid Sequence; D001665:Binding Sites; D002135:Calcium-Binding Proteins / Q000737:chemistry*; D018360:Crystallography, X-Ray; D003598:Cytoskeletal Proteins / Q000737:chemistry*; D016189:Dystrophin / Q000031:analogs & derivatives*; D006801:Humans; D008565:Membrane Proteins / Q000737:chemistry*; D008840:Microfilament Proteins; D008969:Molecular Sequence Data; D010802:Phylogeny; D017433:Protein Structure, Secondary; D011994:Recombinant Proteins / Q000737:chemistry; D016415:Sequence Alignment; D013049:Spectrin / Q000737:chemistry; D049411:Utrophin; D000096985:Calponins
chemical_list: D000199:Actins; D002135:Calcium-Binding Proteins; D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; D008840:Microfilament Proteins; D011994:Recombinant Proteins; D013049:Spectrin; D049411:Utrophin; D000096985:Calponins