Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice

Human Molecular Genetics
Roger A WilliamsonK P Campbell

Abstract

Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC), a protein assembly that plays a critical role in a variety of muscular dystrophies. In order to better understand the function of dystroglycan in development and disease, we have generated a null allele of dystroglycan (Dag1neo2) in mice. Heterozygous Dag1neo2 mice are viable and fertile. In contrast, homozygous Dag1neo2 embryos exhibit gross developmental abnormalities beginning around 6.5 days of gestation. Analysis of the mutant phenotype indicates that an early defect in the development of homozygous Dag1neo2 embryos is a disruption of Reichert's membrane, an extra-embryonic basement membrane. Consistent with the functional defects observed in Reichert's membrane, dystroglycan protein is localized in apposition to this structure in normal egg cylinder stage embryos. We also show that the localization of two critical structural elements of Reichert's membrane--laminin and collagen IV--are specifically disrupted in the homozygous Dag1neo2 embryos. Taken together, the data indicate that dystroglycan is required for the development of Reichert's membrane. Furthermore, these results suggest that disruption of basement membrane organization might be...Continue Reading

References

Jan 1, 1975·Developmental Biology·M A JensenR L Brent
Sep 20, 1991·Cell·J M Ervasti, K P Campbell
Apr 1, 1980·Developmental Biology·I LeivoJ Wartiovaara
Aug 1, 1995·Developmental Dynamics : an Official Publication of the American Association of Anatomists·P L Durham, J M Snyder
Nov 10, 1995·The Journal of Biological Chemistry·D JungK P Campbell
Jul 1, 1995·The Journal of Cell Biology·M DurbeejP Ekblom
Jul 1, 1994·Neuron·J SugiyamaZ W Hall
Aug 1, 1993·The Journal of Cell Biology·J M Ervasti, K P Campbell
Sep 15, 1993·Proceedings of the National Academy of Sciences of the United States of America·A NagyJ C Roder
Oct 1, 1995·Developmental Dynamics : an Official Publication of the American Association of Anatomists·J N SchofieldY H Edwards

❮ Previous
Next ❯

Citations

Jun 8, 2000·Developmental Dynamics : an Official Publication of the American Association of Anatomists·H Colognato, P D Yurchenco
Feb 17, 2001·Muscle & Nerve·R DraviamS C Watkins
Jul 12, 2002·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Heather J SpenceSteven J Winder
Jan 26, 2006·Histochemistry and Cell Biology·Edward J MacarakPamela S Howard
Sep 14, 2006·Journal of Human Genetics·Motoi Kanagawa, Tatsushi Toda
Sep 6, 2008·Glycoconjugate Journal·Christopher J Moore, Jane E Hewitt
Jan 19, 2008·Journal of Molecular Neuroscience : MN·Mariz VainzofLydia U Yamamoto
Mar 5, 2004·Matrix Biology : Journal of the International Society for Matrix Biology·Peter D YurchencoBruce L Patton
Apr 20, 2005·Neurobiology of Disease·Hiroki KurahashiTatsushi Toda
Mar 21, 2003·Biochemical and Biophysical Research Communications·Vianney JayasinhaPaul T Martin
Apr 5, 2003·Journal of Molecular and Cellular Cardiology·Elizabeth McNallyAhlke Heydemann
Sep 28, 1998·Brain Research. Developmental Brain Research·K A DeystJ R Fallon
Feb 17, 2000·Brain Research. Developmental Brain Research·S Lin, J M Burgunder
Feb 17, 1998·Trends in Neurosciences·M A Ruegg, J L Bixby
Feb 17, 2000·Trends in Neurosciences·D J Blake, S Kröger
Sep 6, 2000·Trends in Genetics : TIG·A De Arcangelis, E Georges-Labouesse
Apr 3, 2003·Brain Research. Molecular Brain Research·Timothy R HenionFrances I Smith
Apr 13, 2000·Biology of the Cell·T DarribèreD Alfandari
Nov 6, 2002·Biochimica Et Biophysica Acta·Prabhjit K Grewal, Jane E Hewitt
Dec 5, 1998·Biochimica Et Biophysica Acta·T SasakiT Endo
Dec 2, 1999·Biochimica Et Biophysica Acta·T Endo
Mar 6, 2003·Gene Expression Patterns : GEP·Andrea Lunardi, Luciana Dente
Mar 14, 2001·Matrix Biology : Journal of the International Society for Matrix Biology·M A Ruegg
Apr 26, 2003·Matrix Biology : Journal of the International Society for Matrix Biology·Peter EkblomJan F Talts
Jun 15, 1999·Matrix Biology : Journal of the International Society for Matrix Biology·M Aumailley, P Rousselle
Nov 18, 1998·Current Opinion in Cell Biology·M DurbeejK P Campbell
Oct 6, 1999·Current Opinion in Cell Biology·M D Henry, K P Campbell
Jun 22, 2002·Current Opinion in Genetics & Development·Madeleine Durbeej, Kevin P Campbell
Jun 6, 2003·Current Opinion in Genetics & Development·Isin Dalkilic, Louis M Kunkel
Dec 4, 2001·Neuromuscular Disorders : NMD·Marina CeccariniTamara C Petrucci
Jun 12, 2003·Neuromuscular Disorders : NMD·Vianney JayasinhaPaul T Martin
Dec 18, 2003·Neuromuscular Disorders : NMD·Kiichiro MatsumuraTeruo Shimizu
Feb 13, 2001·Trends in Biochemical Sciences·S J Winder
Aug 26, 2006·Nature Clinical Practice. Neurology·Paul T Martin
Jan 19, 2002·Differentiation; Research in Biological Diversity·M DurbeejP Ekblom

❮ Previous
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