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      Cystic Diseases of the Kidney: Role of Adhesion Molecules in Normal and Abnormal Tubulogenesis

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          Abstract

          This short review summarizes some information concerning what is known about matrix adhesion molecules, focal adhesion proteins, and cell-cell adhesion molecules in normal renal development and cystic diseases of the kidney. The focus is on human nephrogenesis and disease, but utilizes critical information gained from genetically manipulated mouse models. Interestingly, a significant role for the human PKD-1-encoded gene product, polycystin-1, has been found in cell-matrix interactions via integrins during development, and mutations lead to autosomal dominant polycystic kidney disease (ADPKD). Recent studies on human ADPKD have implicated polycystin-1 in the formation of multiprotein complexes containing focal adhesion proteins at the basal cell surface of the normal ureteric bud. Further evidence of a critical role of cell-matrix interactions via focal adhesion complex formation is provided by the development of renal cystic disease in tensin knockout mice.

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          Most cited references 19

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          Epithelial transformation of metanephric mesenchyme in the developing kidney regulated by Wnt-4.

          The kidney has been widely exploited as a model system for the study of tissue inductions regulating vertebrate organogenesis. Kidney development is initiated by the ingrowth of the Wolfian duct-derived ureteric bud into the presumptive kidney mesenchyme. In response to a signal from the ureter, mesenchymal cells condense, aggregate into pretubular clusters and undergo an epithelial conversion generating a simple tubule. This then undergoes morphogenesis and is transformed into the excretory system of the kidney, the nephron. We report here that the expression of Wnt-4, which encodes a secreted glycoprotein, correlates with, and is required for, kidney tubulogenesis. Mice lacking Wnt-4 activity fail to form pretubular cell aggregates; however, other aspects of mesenchymal and ureteric development are unaffected. Thus, Wnt-4 appears to act as an autoinducer of the mesenchyme to epithelial transition that underlies nephron development.
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            Biology of the syndecans: a family of transmembrane heparan sulfate proteoglycans.

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              Absence of integrin alpha 6 leads to epidermolysis bullosa and neonatal death in mice.

              Cell-extracellular matrix interactions have important roles in many biological processes, including embryonic development, growth control and differentiation. Integrins are the principal receptors for extracellular matrix. They are composed of non-covalently associated alpha and beta chains. Integrin alpha 6 can associate with either beta 1 or beta 4 (refs 2,3). Both integrin complexes are receptors for laminins, major components of basement membranes. The distribution of alpha 6 (refs 4-10) as well as studies using function-blocking antibodies have suggested an essential role for this laminin receptor during embryogenesis, in processes such as endoderm migration or kidney tubule formation9. Here we report that, surprisingly, mice lacking the alpha 6 integrin chain develop to birth. However, they die at birth with severe blistering of the skin and other epithelia, a phenotype reminiscent of the human disorder epidermolysis bullosa. Hemidesmosomes are absent in mutant tissue. This absence is likely to result from the lack of alpha 6/beta 4, the only integrin in hemidesmosomes of stratified squamous and transitional epithelia. Mutations in the genes encoding integrin beta 4 and chains of laminin-5 have been implicated in junctional epidermolysis bullosa. Our study provides evidence that some forms of epidermolysis bullosa may originate from defects of the alpha 6 gene.
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                Author and article information

                Journal
                EXN
                Nephron Exp Nephrol
                10.1159/issn.1660-2129
                Cardiorenal Medicine
                S. Karger AG
                978-3-8055-6886-9
                978-3-318-00442-7
                1660-2129
                1999
                April 1999
                23 April 1999
                : 7
                : 2
                : 114-124
                Affiliations
                Mount Sinai School of Medicine, New York, N.Y., USA
                Article
                20592 Exp Nephrol 1999;7:114–124
                10.1159/000020592
                10213865
                © 1999 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Figures: 3, Tables: 2, References: 107, Pages: 11
                Product
                Self URI (application/pdf): https://www.karger.com/Article/Pdf/20592
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