Cell Structure and Function
Online ISSN : 1347-3700
Print ISSN : 0386-7196
ISSN-L : 0386-7196
REVIEW
Mammalian Skeletogenesis and Extracellular Matrix: What can We Learn from Knockout Mice?
Attila AszódiJohn F. BatemanErika GustafssonRay Boot-HandfordReinhard Fässler
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JOURNAL FREE ACCESS

2000 Volume 25 Issue 2 Pages 73-84

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Abstract

Formation of the vertebrate skeleton and the proper functions of bony and cartilaginous elements are determined by extracellular, cell surface and intracellular molecules. Genetic and biochemical analyses of human heritable skeletal disorders as well as the generation of knockout mice provide useful tools to identify the key players of mammalian skeletogenesis. This review summarises our recent work with transgenic animals carrying ablated genes for cartilage extracellular matrix proteins. Some of these mice exhibit a lethal phenotype associated with severe skeletal defects (type II collagen-null, perlecan-null), whereas others show mild (type IX collagen-null) or no skeletal abnormalities (matrilin-1-null, fibromodulin-null, tenascin-C-null). The appropriate human genetic disorders are discussed and contrasted with the knockout mice phenotypes.

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© 2000 by Japan Society for Cell Biology
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