期刊
CURRENT PHARMACEUTICAL DESIGN
卷 15, 期 12, 页码 1277-1294出版社
BENTHAM SCIENCE PUBL LTD
DOI: 10.2174/138161209787846766
关键词
Extracellular matrix; laminin; collagen; nidogen; perlecan; integrin; dystroglycan; agrin; muscular dystrophy; peripheral neuropathy; Alport's syndrome; Goodpasture syndrome; epidermolysis bullosa; Pierson syndrome
资金
- National Institutes of Health [R37-DK36425, R01-NS38469, R01-NS040759, R01-NS064397]
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [R37DK036425] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R21NS064397, R01NS040759, R01NS038469] Funding Source: NIH RePORTER
Basement membranes are sheet-like cell-adherent extracellular matrices that serve as cell substrata and solid-phase 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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