Abstract
Basement membranes are sheet-like cell-adherent extracellular matrices that serve as cell substrata and solidphase agonists, contributing to tissue organization, stability and differentiation. These matrices are assembled as polymers of laminins and type IV collagens that are tethered to nidogens and proteoglycans. They bind to cell surface molecules that include signal-transducing receptors such as the integrins and dystroglycan and form attachments to adjacent connective tissues. The cell receptors, in turn, provide links between the matrix and underlying cytoskeleton. Genetic diseases of basement membrane and associated components, collectively the basement membrane zone, disrupt the extracellular matrix and/or its linkages to affect nerve, muscle, skin, kidney and other tissues. These diseases can arise due to a loss of matrix integrity, adhesion strength and/or receptor-mediated signaling. An understanding of the mechanisms of basement membrane zone assembly and resulting structure can provide insights into the development of normal tissues and the pathogenic mechanisms that underlie diverse disorders.
Keywords: Extracellular matrix, laminin, collagen, nidogen, integrin, dystroglycan, agrin, muscular dystrophy, peripheral neuropathy, Alport's syndrome
Current Pharmaceutical Design
Title: Developmental and Pathogenic Mechanisms of Basement Membrane Assembly
Volume: 15 Issue: 12
Author(s): Peter D. Yurchenco and Bruce L. Patton
Affiliation:
Keywords: Extracellular matrix, laminin, collagen, nidogen, integrin, dystroglycan, agrin, muscular dystrophy, peripheral neuropathy, Alport's syndrome
Abstract: Basement membranes are sheet-like cell-adherent extracellular matrices that serve as cell substrata and solidphase agonists, contributing to tissue organization, stability and differentiation. These matrices are assembled as polymers of laminins and type IV collagens that are tethered to nidogens and proteoglycans. They bind to cell surface molecules that include signal-transducing receptors such as the integrins and dystroglycan and form attachments to adjacent connective tissues. The cell receptors, in turn, provide links between the matrix and underlying cytoskeleton. Genetic diseases of basement membrane and associated components, collectively the basement membrane zone, disrupt the extracellular matrix and/or its linkages to affect nerve, muscle, skin, kidney and other tissues. These diseases can arise due to a loss of matrix integrity, adhesion strength and/or receptor-mediated signaling. An understanding of the mechanisms of basement membrane zone assembly and resulting structure can provide insights into the development of normal tissues and the pathogenic mechanisms that underlie diverse disorders.
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Cite this article as:
Yurchenco D. Peter and Patton L. Bruce, Developmental and Pathogenic Mechanisms of Basement Membrane Assembly, Current Pharmaceutical Design 2009; 15 (12) . https://dx.doi.org/10.2174/138161209787846766
DOI https://dx.doi.org/10.2174/138161209787846766 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
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