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© 1995 Oxford University Press

RESEARCH-ARTICLE

Dp140: a novel 140 kDa CNS transcript from the dystrophin locus

Hart G. W. Lidov*, Sara Selig1,2 and Louis M. Kunkel1,2

Departments of Pathology 300 Longwood Ave, Boston, MA 02115, USA 1Departments of Genetics, Harvard Medical School 300 Longwood Ave, Boston, MA 02115, USA 2Howard Hughes Medical Institute at Children's Hospital 300 Longwood Ave, Boston, MA 02115, USA

*To whom correspondence should be addressed

Received September 19, 1994; Revised December 12, 1994; Accepted December 12, 1994

We have identified a 7.5 kb transcript from the dystrophin locus which encodes a novel 140 kDa protein (Dp140). Based on immunoblotting Dp140 consists of the distal rod domain and C-terminus of 427 kDa dystrophin and is found throughout the CNS. This protein is transcribed from an alternative promoter in the dystrophin locus upstream to exon 45. The unique 5' first exon is conserved between rat and human. The transcript has a 1 kb 5' untranslated region, and the first methionine initiation codon occurs in exon 51, predicting a protein of 140 kDa. Several studies report that Duchenne dystrophy patients with deletions in the exon 45–52 region have an increased incidence of cognitive impairment. Such deletions would atfect expression of 427 kDa dystrophin and this shorter 140 kDa isoform but not the recently described small distal transcripts Dp116 or Dp71, suggesting particular importance to CNS function.


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