Literature DB >> 8186707

Utrophin: a potential replacement for dystrophin?

J M Tinsley1, K E Davies.   

Abstract

This paper reviews the evidence that utrophin, the autosomally encoded protein related to dystrophin, may be capable of performing the same cellular functions as dystrophin. If this is the case, it may be possible to modify the regulation of utrophin expression as an alternative route to dystrophin gene therapy for sufferers of DMD and/or BMD.

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Year:  1993        PMID： 8186707     DOI： 10.1016/0960-8966(93)90111-v

Source DB:  PubMed          Journal:  Neuromuscul Disord        ISSN： 0960-8966            Impact factor:   4.296


  18 in total

Review 1.  Understanding dystrophinopathies: an inventory of the structural and functional consequences of the absence of dystrophin in muscles of the mdx mouse.

Authors:  J M Gillis
Journal:  J Muscle Res Cell Motil       Date:  1999-10       Impact factor: 2.698

Review 2.  Gene replacement therapies for duchenne muscular dystrophy using adeno-associated viral vectors.

Authors:  Jane T Seto; Julian N Ramos; Lindsey Muir; Jeffrey S Chamberlain; Guy L Odom
Journal:  Curr Gene Ther       Date:  2012-06       Impact factor: 4.391

3.  Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin.

Authors:  Dejia Li; Akshay Bareja; Luke Judge; Yongping Yue; Yi Lai; Rebecca Fairclough; Kay E Davies; Jeffrey S Chamberlain; Dongsheng Duan
Journal:  J Cell Sci       Date:  2010-05-18       Impact factor: 5.285

4.  2015 William Allan Award.

Authors:  Kay E Davies
Journal:  Am J Hum Genet       Date:  2016-03-03       Impact factor: 11.025

5.  Specific interaction of the actin-binding domain of dystrophin with intermediate filaments containing keratin 19.

Authors:  Michele R Stone; Andrea O'Neill; Dawn Catino; Robert J Bloch
Journal:  Mol Biol Cell       Date:  2005-07-06       Impact factor: 4.138

Review 6.  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

7.  A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy.

Authors:  E A Burton; J M Tinsley; P J Holzfeind; N R Rodrigues; K E Davies
Journal:  Proc Natl Acad Sci U S A       Date:  1999-11-23       Impact factor: 11.205

8.  The role of basal and myogenic factors in the transcriptional activation of utrophin promoter A: implications for therapeutic up-regulation in Duchenne muscular dystrophy.

Authors:  K J Perkins; E A Burton; K E Davies
Journal:  Nucleic Acids Res       Date:  2001-12-01       Impact factor: 16.971

9.  Duchenne muscular dystrophy gene therapy: Lost in translation?

Authors:  Dongsheng Duan
Journal:  Res Rep Biol       Date:  2011-03

Review 10.  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
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