Targeted inactivation of Dp71, the major non-muscle product of the DMD gene: differential activity of the Dp71 promoter during development |
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Authors: | Sarig R; Mezger-Lallemand V; Gitelman I; Davis C; Fuchs O; Yaffe D; Nudel U |
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Institution: | Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot 76100, Israel. |
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Abstract: | The dystrophin gene, which is defective in Duchenne muscular dystrophy
(DMD), also encodes a number of smaller products controlled by internal
promoters. Dp71, which consists of the two C-terminal domains of
dystrophin, is the most abundant product of the gene in non-muscle tissues
and is the major product in adult brain. To study the possible function of
Dp71 and its expression during development, we specifically inactivated the
expression of Dp71 by replacing its first and unique exon and a part of the
concomitant intron with a beta-galactosidase reporter gene. X-Gal staining
of Dp71-null mouse embryos and tissues revealed a very stage- and cell
type-specific activity of the Dp71 promoter during development and during
differentiation of various tissues, including the nervous system, eyes,
limb buds, lungs, blood vessels, vibrissae and hair follicles. High
activity of the Dp71 promoter often seemed to be associated with
morphogenic events and terminal differentiation. In some tissues the
activity greatly increased towards birth.
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