Expression of the 71 kDa dystrophin isoform (Dp71) evaluated by gene targeting

To investigate the function of the major non-muscle dystrophin isoform, Dp7 1. we substituted a beta-galactosidase (beta gal) reporter gene for Dp71 by homologous recombination in embryonic stem cells. Staining for beta gal ac tivity in chimeric mice revealed Dp71 promoter activity in glial cells in t he CNS, in neurons of the inner nuclear and inner plexiform layers of the r etina, and in the kidney tubules and collecting ducts. Our observations dem onstrate that Dp71 is widely expressed in the adult CNS (retina, cerebellum , cerebral cortex, ependyma, and choroid) as well as the adult kidney epith elium and suggest a broad function for Dp71 in differentiated tissues. (C) 1999 Elsevier Science B.V. All rights reserved.