Utrophin (UTRN)

DRP1; DMDL; DRP; Homologous To Dystrophin; Dystrophin-related protein 1

SPN	Q846
HGNC	12635
MGI	104631
PubMed	9846586
RGD	3947
UniProt	P46939
Wiki	Utrophin

Utrophin is a component of the cytoskeleton. Utrophin was found during research into Duchenne's muscular dystrophy. The name is a contraction for ubiquitous dystrophin. The 900 kb gene for utrophin is found on the long arm of human chromosome 6. Utrophin was discovered due to its homology with dystrophin. It was found by screening a peptide containing the C-terminal domain of dystrophin against cDNA libraries. The homology varies over its full length from less than 30% in regions of the central rod structural domain, to 85% (identity 73%) for the actin binding domain.The tertiary structure of utrophin contains a C-terminus that consists of protein-protein interaction motifs that interact with dystroglycan, a central rod region consisting of a triple coiled-coil repeat, and an actin-binding N-terminus.

Reference

"Primary structure of dystrophin-related protein."
Nature 360:591-593(1992) [PubMed] [Europe PMC] [Abstract]

"Up71 and up140, two novel transcripts of utrophin that are homologues of short forms of dystrophin."
Hum. Mol. Genet. 8:1271-1278(1999) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and analysis of human chromosome 6."
Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]

"A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy."
Proc. Natl. Acad. Sci. U.S.A. 96:14025-14030(1999) [PubMed] [Europe PMC] [Abstract]

"Syntrophin binds to an alternatively spliced exon of dystrophin."
J. Cell Biol. 128:363-371(1995) [PubMed] [Europe PMC] [Abstract]

"The three human syntrophin genes are expressed in diverse tissues, have distinct chromosomal locations, and each bind to dystrophin and its relatives."
J. Biol. Chem. 271:2724-2730(1996) [PubMed] [Europe PMC] [Abstract]

"Contribution of the different modules in the utrophin carboxy-terminal region to the formation and regulation of the DAP complex."
FEBS Lett. 471:229-234(2000) [PubMed] [Europe PMC] [Abstract]

"Adhesion-dependent tyrosine phosphorylation of (beta)-dystroglycan regulates its interaction with utrophin."
J. Cell Sci. 113:1717-1726(2000) [PubMed] [Europe PMC] [Abstract]

"Interactions of intermediate filament protein synemin with dystrophin and utrophin."
Biochem. Biophys. Res. Commun. 346:768-777(2006) [PubMed] [Europe PMC] [Abstract]

"Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."
Cell 127:635-648(2006) [PubMed] [Europe PMC] [Abstract]

"A quantitative atlas of mitotic phosphorylation."
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]

"Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]

"Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"The actin binding affinity of the utrophin tandem calponin-homology domain is primarily determined by its N-terminal domain."
Biochemistry 53:1801-1809(2014) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"The 2.0-A structure of the second calponin homology domain from the actin-binding region of the dystrophin homologue utrophin."
J. Mol. Biol. 285:1257-1264(1999) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of the actin-binding region of utrophin reveals a head-to-tail dimer."
Structure 7:1539-1546(1999) [PubMed] [Europe PMC] [Abstract]

"Structure of the utrophin actin-binding domain bound to F-actin reveals binding by an induced fit mechanism."
J. Mol. Biol. 297:465-480(2000) [PubMed] [Europe PMC] [Abstract]