Literature DB >> 8640231

Characterization of DRP2, a novel human dystrophin homologue.

R G Roberts1, T C Freeman, E Kendall, D L Vetrie, A K Dixon, C Shaw-Smith, Q Bone, M Bobrow.   

Abstract

The currently recognised dystrophin protein family comprises the archetype, dystrophin, its close relative, utrophin or dystrophin-related protein (DRP), and a distantly related protein known as the 87K tyrosine kinase substrate. During the course of a phylogenetic study of sequences encoding the characteristic C-terminal domains of dystrophin-related proteins, we identified an unexpected novel class of vertebrate dystrophin-related sequences. We term this class dystrophin-related protein 2 (DRP2), and suggest that utrophin/DRP be renamed DRP1 to simplify future nomenclature. DRP2 is a relatively small protein, encoded in man by a 45 kb gene localized to Xq22. It is expressed principally in the brain and spinal cord, and is similar in overall structure to the Dp116 dystrophin isoform. The discovery of a novel relative of dystrophin substantially broadens the scope for study of this interesting group of proteins and their associated glycoprotein complexes.

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Year:  1996        PMID: 8640231     DOI: 10.1038/ng0696-223

Source DB:  PubMed          Journal:  Nat Genet        ISSN: 1061-4036            Impact factor:   38.330


  21 in total

1.  Presence of invertebrate dystrophin-like products in obliquely striated muscle of the leech, Pontobdella muricata (Annelida, Hirudinea).

Authors:  M Royuela; R Paniagua; F Rivier; G Hugon; A Robert; D Mornet
Journal:  Histochem J       Date:  1999-09

Review 2.  The membrane-cytoskeleton interface: the role of dystrophin and utrophin.

Authors:  S J Winder
Journal:  J Muscle Res Cell Motil       Date:  1997-12       Impact factor: 2.698

3.  Sequence analysis of LRPPRC and its SEC1 domain interaction partners suggests roles in cytoskeletal organization, vesicular trafficking, nucleocytosolic shuttling, and chromosome activity.

Authors:  Leyuan Liu; Wallace L McKeehan
Journal:  Genomics       Date:  2002-01       Impact factor: 5.736

Review 4.  Viral-mediated gene therapy for the muscular dystrophies: successes, limitations and recent advances.

Authors:  Guy L Odom; Paul Gregorevic; Jeffrey S Chamberlain
Journal:  Biochim Biophys Acta       Date:  2006-09-26

5.  Activation of utrophin promoter by heregulin via the ets-related transcription factor complex GA-binding protein alpha/beta.

Authors:  T S Khurana; A G Rosmarin; J Shang; T O Krag; S Das; S Gammeltoft
Journal:  Mol Biol Cell       Date:  1999-06       Impact factor: 4.138

Review 6.  Dystrophins, utrophins, and associated scaffolding complexes: role in mammalian brain and implications for therapeutic strategies.

Authors:  Caroline Perronnet; Cyrille Vaillend
Journal:  J Biomed Biotechnol       Date:  2010-06-17

Review 7.  Therapeutics for Duchenne muscular dystrophy: current approaches and future directions.

Authors:  Sasha Bogdanovich; Kelly J Perkins; Thomas O B Krag; Tejvir S Khurana
Journal:  J Mol Med (Berl)       Date:  2003-12-12       Impact factor: 4.599

8.  Absence of Dystrophin Related Protein-2 disrupts Cajal bands in a patient with Charcot-Marie-Tooth disease.

Authors:  Kathryn M Brennan; Yunhong Bai; Chiara Pisciotta; Suola Wang; Shawna M E Feely; Mark Hoegger; Laurie Gutmann; Steven A Moore; Michael Gonzalez; Diane L Sherman; Peter J Brophy; Stephan Züchner; Michael E Shy
Journal:  Neuromuscul Disord       Date:  2015-07-07       Impact factor: 4.296

9.  Profound human/mouse differences in alpha-dystrobrevin isoforms: a novel syntrophin-binding site and promoter missing in mouse and rat.

Authors:  Sabrina V Böhm; Panayiotis Constantinou; Sipin Tan; Hong Jin; Roland G Roberts
Journal:  BMC Biol       Date:  2009-12-04       Impact factor: 7.431

Review 10.  The roles of the dystrophin-associated glycoprotein complex at the synapse.

Authors:  Gonneke S K Pilgram; Saranyapin Potikanond; Richard A Baines; Lee G Fradkin; Jasprina N Noordermeer
Journal:  Mol Neurobiol       Date:  2009-11-09       Impact factor: 5.590

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