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Human Molecular Genetics, 2003, Vol. 12, No. 6 601-615
© 2003 Oxford University Press

The dystrophin associated protein complex in zebrafish

J. R. Guyon1,2,3, A. N. Mosley1, Y. Zhou2,4, K. F. O'Brien1,2, X. Sheng4, K. Chiang4, A. J. Davidson3,4, J. M. Volinski1,3, L. I. Zon2,3,4 and L. M. Kunkel1,2,3,*

1Division of Genetics, Children's Hospital, Boston, MA, USA, 2Department of Genetics, Harvard Medical School, Boston, MA, USA, 3Howard Hughes Medical Institute, Boston, MA, USA and 4Division of Hematology/Oncology, Children's Hospital Boston, MA, USA

Received October 21, 2002; Accepted January 22, 2003

Many cases of muscular dystrophy in humans are caused by mutations in members of the dystrophin associated protein complex (DAPC). Zebrafish are small vertebrates whose bodies are composed predominantly of skeletal muscle, making them attractive models for studying mammalian muscle disorders. Potential orthologs to most of the human DAPC proteins have been found in zebrafish by database screening. Expression of the sarcoglycans, dystroglycan and dystrophin has been confirmed by western blotting. Immunohistochemical and biochemical techniques localize these proteins to the muscle cell membrane in adult zebrafish. Morpholino (MO) experiments designed to inhibit the translation of dystrophin mRNA produce juvenile zebrafish that are less active than zebrafish injected with control morpholinos. Western blot analysis of the dystrophin morpholino-injected zebrafish shows concurrent reduction of dystrophin and the sarcoglycans, suggesting that these proteins, like those in mammals, are part of a complex whose integrity is dependant on dystrophin expression. These results indicate that the zebrafish is an excellent animal model in which to approach the study of dystrophin and its associated proteins.

* To whom correspondence should be addressed at: Division of Genetics, Children's Hospital Boston, Enders Rm 570, 300 Longwood Ave, Boston, MA 02115, USA. Tel:+1 6173558200; Email: kunkel{at}enders.tch.harvard.edu


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