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Gene Review

UTRN  -  utrophin

Homo sapiens

Synonyms: DMDL, DRP, DRP-1, DRP1, Dystrophin-related protein 1, ...
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Disease relevance of UTRN


Psychiatry related information on UTRN

  • CONCLUSIONS: Two DRPs, needs additional drug therapy and wrong drug, are associated with reduced self-reported physical health in arthritis and low back pain, while the DRP needs additional drug therapy is also associated with reduced self-reported mental health [5].
  • All subjects were interviewed by psychiatrists and were administered psychological tests such as SAS (self-rating anxiety scale), SDS (self-rating depression scale), POMS (Profile of Mood States), HADS (Hospital Anxiety and Depression Scale) and DRP (Depression-related personality traits) [6].

High impact information on UTRN


Chemical compound and disease context of UTRN


Biological context of UTRN


Anatomical context of UTRN


Associations of UTRN with chemical compounds


Physical interactions of UTRN


Enzymatic interactions of UTRN


Regulatory relationships of UTRN

  • Utrophin is therefore expressed in the presence and absence of dystrophin and down-regulated before birth in normal fetal muscle fibres [25].

Other interactions of UTRN

  • The cardiac expression of the dystrophin related protein utrophin was increased, and the 43 kDa (beta-dystroglycan), 50 kDa (alpha-sarcoglycan) and 59 kDa (syntrophin) dystrophin associated proteins (DAPs) were co-isolated and present in nearly normal amounts in the membrane [26].
  • The staining intensities of utrophin, vimentin and desmin were also higher in small regenerating fibers [27].
  • We found no correlation between utrophin expression and DAP expression [28].
  • 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 [29].
  • In Becker dystrophy, 43DAG expression was more extensive around individual fibres, showed more interfibre variation and was more closely related to the intensity of immunolabelling for both dystrophin and utrophin than in Duchenne dystrophy [30].
  • Laser-capture microscopy of tibialis anterior NMJ and extrasynaptic transcriptomes and gene transfer studies provide spatial and direct evidence, respectively, for ERF-mediated utrophin repression in vivo [31].

Analytical, diagnostic and therapeutic context of UTRN


  1. The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain. Khurana, T.S., Watkins, S.C., Kunkel, L.M. J. Cell Biol. (1992) [Pubmed]
  2. Alternative splicing of dystrobrevin regulates the stoichiometry of syntrophin binding to the dystrophin protein complex. Newey, S.E., Benson, M.A., Ponting, C.P., Davies, K.E., Blake, D.J. Curr. Biol. (2000) [Pubmed]
  3. Localization and quantitation of the chromosome 6-encoded dystrophin-related protein in normal and pathological human muscle. Karpati, G., Carpenter, S., Morris, G.E., Davies, K.E., Guerin, C., Holland, P. J. Neuropathol. Exp. Neurol. (1993) [Pubmed]
  4. Utrophin, the autosomal homologue of dystrophin, is widely-expressed and membrane-associated in cultured cell lines. Nguyen, T.M., Le, T.T., Blake, D.J., Davies, K.E., Morris, G.E. FEBS Lett. (1992) [Pubmed]
  5. Drug-related problems and quality of life in arthritis and low back pain sufferers. Ernst, M.E., Iyer, S.S., Doucette, W.R. Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research. (2003) [Pubmed]
  6. Depression among cancer patients. Hosaka, T., Aoki, T. Psychiatry and clinical neurosciences. (1996) [Pubmed]
  7. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Blake, D.J., Weir, A., Newey, S.E., Davies, K.E. Physiol. Rev. (2002) [Pubmed]
  8. A role for dystrophin-associated glycoproteins and utrophin in agrin-induced AChR clustering. Campanelli, J.T., Roberds, S.L., Campbell, K.P., Scheller, R.H. Cell (1994) [Pubmed]
  9. Inactivation of digoxin by the gut flora: reversal by antibiotic therapy. Lindenbaum, J., Rund, D.G., Butler, V.P., Tse-Eng, D., Saha, J.R. N. Engl. J. Med. (1981) [Pubmed]
  10. An autosomal transcript in skeletal muscle with homology to dystrophin. Love, D.R., Hill, D.F., Dickson, G., Spurr, N.K., Byth, B.C., Marsden, R.F., Walsh, F.S., Edwards, Y.H., Davies, K.E. Nature (1989) [Pubmed]
  11. Dystrophin-related protein in skeletal muscles in neuromuscular disorders: immunohistochemical study. Takemitsu, M., Nonaka, I., Sugita, H. Acta Neuropathol. (1993) [Pubmed]
  12. Effects of ACE inhibitor and AT1 blocker on dystrophin-related proteins and calpain in failing heart. Takahashi, M., Tanonaka, K., Yoshida, H., Oikawa, R., Koshimizu, M., Daicho, T., Toyo-Oka, T., Takeo, S. Cardiovasc. Res. (2005) [Pubmed]
  13. The utrophin and dystrophin genes share similarities in genomic structure. Pearce, M., Blake, D.J., Tinsley, J.M., Byth, B.C., Campbell, L., Monaco, A.P., Davies, K.E. Hum. Mol. Genet. (1993) [Pubmed]
  14. Dystrophin and dystrophin-related proteins: a review of protein and RNA studies. Love, D.R., Byth, B.C., Tinsley, J.M., Blake, D.J., Davies, K.E. Neuromuscul. Disord. (1993) [Pubmed]
  15. Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines. Nguyen, T.M., Ellis, J.M., Love, D.R., Davies, K.E., Gatter, K.C., Dickson, G., Morris, G.E. J. Cell Biol. (1991) [Pubmed]
  16. Characterisation of alpha-dystrobrevin in muscle. Nawrotzki, R., Loh, N.Y., Ruegg, M.A., Davies, K.E., Blake, D.J. J. Cell. Sci. (1998) [Pubmed]
  17. The effect of glucocorticoids on the accumulation of utrophin by cultured normal and dystrophic human skeletal muscle satellite cells. Pasquini, F., Guerin, C., Blake, D., Davies, K., Karpati, G., Holland, P. Neuromuscul. Disord. (1995) [Pubmed]
  18. Laminin-sulfatide binding initiates basement membrane assembly and enables receptor signaling in Schwann cells and fibroblasts. Li, S., Liquari, P., McKee, K.K., Harrison, D., Patel, R., Lee, S., Yurchenco, P.D. J. Cell Biol. (2005) [Pubmed]
  19. Adhesion-dependent tyrosine phosphorylation of (beta)-dystroglycan regulates its interaction with utrophin. James, M., Nuttall, A., Ilsley, J.L., Ottersbach, K., Tinsley, J.M., Sudol, M., Winder, S.J. J. Cell. Sci. (2000) [Pubmed]
  20. Utrophin lacks the rod domain actin binding activity of dystrophin. Amann, K.J., Guo, A.W., Ervasti, J.M. J. Biol. Chem. (1999) [Pubmed]
  21. The carboxyterminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex. Buechler, C., Boettcher, A., Bared, S.M., Probst, M.C., Schmitz, G. Biochem. Biophys. Res. Commun. (2002) [Pubmed]
  22. Contribution of the different modules in the utrophin carboxy-terminal region to the formation and regulation of the DAP complex. Tommasi di Vignano, A., Di Zenzo, G., Sudol, M., Cesareni, G., Dente, L. FEBS Lett. (2000) [Pubmed]
  23. Dephosphorylation of beta2-syntrophin and Ca2+/mu-calpain-mediated cleavage of ICA512 upon stimulation of insulin secretion. Ort, T., Voronov, S., Guo, J., Zawalich, K., Froehner, S.C., Zawalich, W., Solimena, M. EMBO J. (2001) [Pubmed]
  24. MARCH-V is a novel mitofusin 2- and Drp1-binding protein able to change mitochondrial morphology. Nakamura, N., Kimura, Y., Tokuda, M., Honda, S., Hirose, S. EMBO Rep. (2006) [Pubmed]
  25. Dystrophin-related protein, utrophin, in normal and dystrophic human fetal skeletal muscle. Clerk, A., Morris, G.E., Dubowitz, V., Davies, K.E., Sewry, C.A. Histochem. J. (1993) [Pubmed]
  26. A 5' dystrophin duplication mutation causes membrane deficiency of alpha-dystroglycan in a family with X-linked cardiomyopathy. Bies, R.D., Maeda, M., Roberds, S.L., Holder, E., Bohlmeyer, T., Young, J.B., Campbell, K.P. J. Mol. Cell. Cardiol. (1997) [Pubmed]
  27. Relationship between utrophin and regenerating muscle fibers in duchenne muscular dystrophy. Shim, J.Y., Kim, T.S. Yonsei Med. J. (2003) [Pubmed]
  28. Dystrophin-associated protein abnormalities in dystrophin-deficient muscle fibers from symptomatic and asymptomatic Duchenne/Becker muscular dystrophy carriers. Di Blasi, C., Morandi, L., Barresi, R., Blasevich, F., Cornelio, F., Mora, M. Acta Neuropathol. (1996) [Pubmed]
  29. The 2.0 A structure of the second calponin homology domain from the actin-binding region of the dystrophin homologue utrophin. Keep, N.H., Norwood, F.L., Moores, C.A., Winder, S.J., Kendrick-Jones, J. J. Mol. Biol. (1999) [Pubmed]
  30. Expression of the 43 kDa dystrophin-associated glycoprotein in human neuromuscular disease. Helliwell, T.R., Nguyen, T.M., Morris, G.E. Neuromuscul. Disord. (1994) [Pubmed]
  31. Ets-2 repressor factor silences extrasynaptic utrophin by N-box mediated repression in skeletal muscle. Perkins, K.J., Basu, U., Budak, M.T., Ketterer, C., Baby, S.M., Lozynska, O., Lunde, J.A., Jasmin, B.J., Rubinstein, N.A., Khurana, T.S. Mol. Biol. Cell (2007) [Pubmed]
  32. Dystrophin and related proteins. Tinsley, J.M., Blake, D.J., Pearce, M., Knight, A.E., Kendrick-Jones, J., Davies, K.E. Curr. Opin. Genet. Dev. (1993) [Pubmed]
  33. Utility of dystrophin and utrophin staining in childhood muscular dystrophy. Sundaram, C., Vydehi, B., Meena, K., Murthy, J. Indian journal of pathology & microbiology. (2004) [Pubmed]
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