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Primary structure of dystrophin-related protein

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Abstract

DYSTROPHIN-RELATED protein (DRP or 'utrophin'1) is localized in normal adult muscle primarily at the neuromuscular junction2–4. In the absence of dystrophin in Duchenne muscular dystrophy (DMD) patients, DRP is also present in the sarcolemma3–7. DRP is expressed in fetal and regenerating muscle and may play a similar role to dystrophin in early development3,7–9, although it remains to be determined whether DRP can functionally replace dystrophin in adult tissue. Previously we described a 3.5-kilobase complementary DNA clone that exhibits 80 per cent homology to the C-terminal domain of dystrophin10. This sequence identifies a 13-kilobase transcript that maps to human chromosome 6 (refs 2, 11). Antibodies raised against the gene product identify a polypeptide with a relative molecular mass of about 400K in all tissues examined7,8,12. To investigate the relationship between DRP and dystrophin in more detail, we have cloned and sequenced the whole DRP cDNA. Homology between DRP and dystrophin extends over their entire length, suggesting that they derive from a common ancestral gene. Comparative analysis of primary sequences highlights regions of functional importance, including those that may mediate the localization of DRP and dystrophin in the muscle cell.

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Author notes

  1. D. R. Love

    Present address: CRC Human Cancer Genetics Research Group, Department of Pathology, University of Cambridge, Tennis Court Road, Cambridge, CB2 1QP, UK

  2. B. C. Byth

    Present address: The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada

  3. J. M. Tinsley, D. J. Blake, A. Roche, U. Fairbrother, J. Riss, B. C. Byth, A. E. Knight, J. Kendrick-Jones, G. K. Suthers, D. R. Love, Y. H. Edwards and K. E. Davies: Institute of Molecular Medicine, John Radcliffe Hospital, Headington,

  4. U. Fairbrother and Y. H. Edwards: Galton Laboratory, MRC Human Biochemical Genetics Laboratory, Wolfson House, 4 Stephenson Way, London NW1 2HE, UK

  5. A. E. Knight and J. Kendrick-Jones: MRC Laboratory of Molecular Biology, Hills Road, Cambridge CB2 2QH, UK

  6. K. E. Davies: To whom correspondence should be addressed.

Authors and Affiliations

  1. Oxford, 0X3 9DU, UK

    J. M. Tinsley, D. J. Blake, A. Roche, U. Fairbrother, J. Riss, B. C. Byth, A. E. Knight, J. Kendrick-Jones, G. K. Suthers, D. R. Love, Y. H. Edwards & K. E. Davies

  2. CRC Human Cancer Genetics Research Group, Department of Pathology, University of Cambridge, Tennis Court Road, Cambridge, CB2 1QP, UK

    B. C. Byth

  3. The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada

    D. R. Love

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  1. J. M. Tinsley
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  2. D. J. Blake
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  3. A. Roche
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  4. U. Fairbrother
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  5. J. Riss
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  6. B. C. Byth
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  7. A. E. Knight
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  8. J. Kendrick-Jones
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  9. G. K. Suthers
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  10. D. R. Love
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  11. Y. H. Edwards
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  12. K. E. Davies
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Tinsley, J., Blake, D., Roche, A. et al. Primary structure of dystrophin-related protein. Nature 360, 591–593 (1992). https://doi.org/10.1038/360591a0

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  • DOI: https://doi.org/10.1038/360591a0

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