Perkins Kelly J, Davies Kay E
Department of Human Anatomy and Genetics, University of Oxford, South Parks Road, Oxford OX1 3QU, UK.
FEBS Lett. 2003 Mar 13;538(1-3):168-72. doi: 10.1016/s0014-5793(03)00175-3.
Duchenne muscular dystrophy is caused by dystrophin deficiency, which can be prevented in the mdx mouse model by over-expression of an autosomal homologue, utrophin. Utrophin has two characterised full-length promoters, A and B. No data are available on the transcriptional regulation of B utrophin, which has been recently localised to the endothelium. Similar to characterised endothelial promoters, Ets and Ap-1 individually trans-activate the human B core promoter. Synergistic activation by GATA-2 and c-jun to the order of 20-fold was observed.