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Transient expression of Dp140, a product of the Duchenne muscular dystrophy locus, during kidney tubulogenesis

Durbeej, Madelaine LU ; Jung, Daniel ; Hjalt, Tord LU ; Campbell, Kevin P. and Ekblom, Peter LU (1997) In Developmental Biology 181(2). p.67-156
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. By in situ hybridization 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... (More)

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. By in situ hybridization 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 VIA4(2)A3 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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author
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publishing date
type
Contribution to journal
publication status
published
subject
keywords
Animals, Antibodies, Monoclonal/immunology, Cytoskeletal Proteins/metabolism, Dystroglycans, Dystrophin/biosynthesis, Epitopes/immunology, Gene Expression Regulation, Developmental, Humans, In Situ Hybridization, Kidney Tubules/embryology, Membrane Glycoproteins/metabolism, Mice, Microscopy, Fluorescence, Molecular Sequence Data, Molecular Weight, Muscular Dystrophies/genetics, Organ Culture Techniques, Organ Specificity, Polymerase Chain Reaction, Protein Binding, RNA, Messenger/biosynthesis, Rats
in
Developmental Biology
volume
181
issue
2
pages
12 pages
publisher
Elsevier
external identifiers
  • scopus:0031568332
  • pmid:9013927
ISSN
0012-1606
DOI
10.1006/dbio.1996.8430
language
English
LU publication?
no
id
d121a3e8-5a1a-4b92-aff6-a56976b6661f
date added to LUP
2023-11-16 11:34:21
date last changed
2024-02-29 21:04:53
@article{d121a3e8-5a1a-4b92-aff6-a56976b6661f,
  abstract     = {{<p>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. By in situ hybridization 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 VIA4(2)A3 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.</p>}},
  author       = {{Durbeej, Madelaine and Jung, Daniel and Hjalt, Tord and Campbell, Kevin P. and Ekblom, Peter}},
  issn         = {{0012-1606}},
  keywords     = {{Animals; Antibodies, Monoclonal/immunology; Cytoskeletal Proteins/metabolism; Dystroglycans; Dystrophin/biosynthesis; Epitopes/immunology; Gene Expression Regulation, Developmental; Humans; In Situ Hybridization; Kidney Tubules/embryology; Membrane Glycoproteins/metabolism; Mice; Microscopy, Fluorescence; Molecular Sequence Data; Molecular Weight; Muscular Dystrophies/genetics; Organ Culture Techniques; Organ Specificity; Polymerase Chain Reaction; Protein Binding; RNA, Messenger/biosynthesis; Rats}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{2}},
  pages        = {{67--156}},
  publisher    = {{Elsevier}},
  series       = {{Developmental Biology}},
  title        = {{Transient expression of Dp140, a product of the Duchenne muscular dystrophy locus, during kidney tubulogenesis}},
  url          = {{http://dx.doi.org/10.1006/dbio.1996.8430}},
  doi          = {{10.1006/dbio.1996.8430}},
  volume       = {{181}},
  year         = {{1997}},
}