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Publication : Dystrophins in vertebrates and invertebrates.

First Author  Roberts RG Year  1998
Journal  Hum Mol Genet Volume  7
Issue  4 Pages  589-95
PubMed ID  9499411 Mgi Jnum  J:46817
Mgi Id  MGI:1202111 Doi  10.1093/hmg/7.4.589
Citation  Roberts RG, et al. (1998) Dystrophins in vertebrates and invertebrates. Hum Mol Genet 7(4):589-95
abstractText  Members of the dystrophin family of proteins perform a critical but incompletely characterized role in the maintenance of membrane-associated complexes at points of intercellular contact in many vertebrate cell types. They interact with, amongst others, the transmembrane laminin receptor dystroglycan, cytoskeletal actin and, indirectly, the intracellular membrane-associated signalling enzyme neuronal nitric oxide synthase (nNOS). Here we describe sequences of a range of dystrophin-related proteins from vertebrate and invertebrate animals (including the important model organism Drosophila melanogaster) and infer an evolutionary history of this family and its relationship to the distantly related dystrobrevins. It appears that most metazoa possess sequences encoding a single highly conserved dystrophin-like protein in addition to a presumed distinct dystrobrevin, derived from an early duplication of an ancestral gene. In the vertebrates (but not the protochordate Amphioxus), the single invertebrate dystrophin-like gene has undergone serial duplication to generate at least three distinct genes encoding proteins which have adopted specialized roles. It is hoped that this broadening of the biology of the dystrophins will afford further opportunities for the advancement of our understanding of the fundamental defect underlying the variety of human genetic disorders which result from aberrant or absent dystrophin-associated complexes.
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