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© 1993 Oxford University Press

RESEARCH-ARTICLE

The utrophin and dystrophin genes share similarities in genomic structure

Marcela Pearce, Derek J. Blake, Jonathon M. Tinsley, Barbara C. Byth+, Louise Campbell, Anthony P. Monaco1 and Kay E. Davies*

Molecular Genetics Group, Institute of Molecular Medicine 1Gene Mapping Group, Imperial Cancer Research Fund Laboratories, Institute of Molecular Medicine, John Radcliffe Hospital Headington, Oxford 0X3 9DU, UK

*To whom correspondence should be addressed

Received September 6, 1993; Revised September 17, 1993; Accepted September 17, 1993

Utrophin and dystrophin are highly homologous proteins which are reciprocally expressed in DMD (Duchenne muscular dystrophy) muscle. The remarkable similarity of these proteins suggests that they may play a similar cellular role in some circumstances; If this were the case then utrophin may be capable of replacing dystrophin in DMD patients. In this paper we show that the genomic structure of the utrophin gene is similar to the dystrophin gene, further exemplifying the relatedness of the two genes and their gene products. We have constructed a 1.25Mb contig of eight yeast artificial chromosome (YAC) clones covering the utrophin gene located on chromosome 6q24. Utrophin is encoded by multiple small exons spanning approximately 900kb. The distribution of exons within the genomic DNA has similarities to that of the dystrophin gene. In contrast to dystrophin, the utrophin gene has a long 5' untranslated region composed of two exons and a cluster of unmethylated, rare-cutting restriction enzyme sites at the 5' end of the gene. Similarities between the genomic structure suggest that utrophin and dystrophin arose through an ancient duplication event involving a large region of genomic DNA.

+Present address: Department of Genetics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada


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