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© 1994 Oxford University Press

RESEARCH-ARTICLE

Apo-dystrophin-1 and apo-dystrophin-2, products of the Duchenne muscular dystrophy locus: expression during mouse embryogenesis and in cultured cell lines

Julian N.Schofield, Derek J.Blake1, Catherine Simmons2, Glenn E.Morris2, Jonathon M.TInsley1, Kay E. Davies1 and Yvonne H. Edwards*

MRC, Human Biochemical Genetics Group, University College London Wotfson House, 4 Stephenson Way, London NW1 2HE, 1Molecular Genetics Group, Institute of Molecular Medicine, John Radcliffe Hospital Oxford 0X3 9DU 2Research Division, N E. Wales Institute Deeside, Clwyd CH5 4BR, UK

Received April 4, 1994; Accepted June 16, 1994

Two promoters In the distal half of the Duchenne Muscular Dystrophy gene drive transcription of mRNAs which have novel first exons and encode the shortened forms of dystrophin, apo-dystrophln-1 (Dp71) and apo-dystrophln-2 (Dp1 16). Apo-dystrophln-1 has a G + C rich promoter and Is expressed in a wide range of cell types, whilst apo-dystrophin-2 is confined to peripheral nerve and brain. We have isolated and sequenced the unique 5‘ exon of rat apo-dystrophln-2 mRNA. Conceptual translation of this sequence Indicates that apo-dystrophin-2 contains a unique 23 amlno acid terminal peptide. Using specific probes derived from sequences at the 5’ ends of apo-dystrophln-1 and apo-dystrophln-2 we have determined the expression of these two mRNAs during mouse embryonic development by RNA In situ hybridization. In contrast to full-length dystrophin, neither of these short dystrophin transcripts appear before organogenesis is well established. Apo-dystrophin-1 mRNA is detected in midllne cells of the ventral neural tube and later, In the ependymal cells lining the ventricles of the brain. These results suggest that apo-dystrophin-1 mRNA is associated with glial cells in the CNS. Apo-dystrophln-1 transcripts are also abundant in the teeth primordla throughout their development. In contrast apo-dystrophin-2 mRNA is largely undetectable during development, although transcripts are seen in the newborn brain. Western blots of late human fetal tissue extracts confirm that apo-dystrophin-2 is most abundant in brain and analysis of RNA and protein in cultured cell lines reveal expression of apo-dystrophln-1 and apo-dystrophin-2 in glioma cells.

*To whom correspondence should be addressed


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