期刊
GLYCOCONJUGATE JOURNAL
卷 19, 期 4-5, 页码 263-267出版社
SPRINGER
DOI: 10.1023/A:1025340215261
关键词
heparan sulfate proteoglycan; cartilage development; mutations; chondrodysplasia; myotonia
Perlecan, a large heparan sulfate proteoglycan (HSPG), is present in the basement membrane and other extracellular matrices. Its protein core is 400 kDa in size and consists of five distinct structural domains. A number of in vitro studies suggest multiple functions of perlecan in cell growth and differentiation and tissue organization. Recent studies with gene knockout mice and human diseases revealed critical in vivo roles of perlecan in cartilage development and neuromuscular junction activity.
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