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Transient Expression of Dp140, a Product of the Duchenne Muscular Dystrophy Locus, during Kidney Tubulogenesis
Journal article   Open access   Peer reviewed

Transient Expression of Dp140, a Product of the Duchenne Muscular Dystrophy Locus, during Kidney Tubulogenesis

Madeleine Durbeej, Daniel Jung, Tord Hjalt, Kevin P Campbell and Peter Ekblom
Developmental biology, Vol.181(2), pp.156-167
01/15/1997
DOI: 10.1006/dbio.1996.8430
PMID: 9013927
url
https://doi.org/10.1006/dbio.1996.8430View
Published (Version of record) Open Access

Abstract

Dystroglycan is a cell surface complex which in muscle links the extracellular matrix protein laminin-2 to the membrane associated cytoskeletal protein dystrophin. Recently it was found that dystroglycan is also expressed in developing epithelial cells. Moreover, antibodies against dystroglycan can perturb epithelial cell development in kidney organ culture. Dystroglycan could provide a link between the basement membrane and the intracellular space also in epithelial cells. However, there is no dystrophin in epithelial cells. Byin situhybridization here we show prominent expression of a shorter isoform of dystrophin, Dp140, in embryonic kidney tubules. In addition, another isoform, Dp71, is expressed by all studied embryonic epithelial cells. Both isoforms share the dystroglycan-binding region of dystrophin but lack the region known to bind to actin. Here we also characterized monoclonal antibodies against different domains of dystrophin and used these to study the distribution of Dp140 protein. In embryonic kidney tubules the dystrophin antibody VIA42A3 stained an intracellular antigen close to the basal cells. In contrast, no staining was observed in adult kidney. We suggest that Dp140 is a structural component during kidney tubulogenesis but it may also be involved in signal transduction.

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