title: Expression of the 71 kDa dystrophin isoform (Dp71) evaluated by gene targeting.
abstract: To investigate the function of the major non-muscle dystrophin isoform, Dp71, we substituted a beta-galactosidase (betagal) reporter gene for Dp71 by homologous recombination in embryonic stem cells. Staining for betagal activity in chimeric mice revealed Dp71 promoter activity in glial cells in the CNS, in neurons of the inner nuclear and inner plexiform layers of the retina, and in the kidney tubules and collecting ducts. Our observations demonstrate that Dp71 is widely expressed in the adult CNS (retina, cerebellum, cerebral cortex, ependyma, and choroid) as well as the adult kidney epithelium and suggest a broad function for Dp71 in differentiated tissues.
authors.affiliation: Department of Human Genetics, University of Michigan Medical School, Ann Arbor, MI 48109-0618, USA.
authors.forename: C N
authors.initials: CN
authors.lastname: Lumeng
authors.forename: M
authors.initials: M
authors.lastname: Hauser
authors.forename: V
authors.initials: V
authors.lastname: Brown
authors.forename: J S
authors.initials: JS
authors.lastname: Chamberlain
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism; D002678:Chimera; D016189:Dystrophin / Q000031:analogs & derivatives* / Q000235:genetics; D005069:Evaluation Studies as Topic; D005786:Gene Expression Regulation / Q000502:physiology*; D018390:Gene Targeting; D017930:Genes, Reporter; D017403:In Situ Hybridization; D051379:Mice; D008810:Mice, Inbred C57BL; D008970:Molecular Weight; D020033:Protein Isoforms / Q000235:genetics*; D001616:beta-Galactosidase / Q000235:genetics*
chemical_list: D016189:Dystrophin; D020033:Protein Isoforms; C085339:apo-dystrophin 1; D001616:beta-Galactosidase