Role of perlecan in skeletal development and diseases.
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.
Hassell
John
J
The Center for Research in Skeletal Development and Pediatric Orthopaedics, Shriners Hospitals for Children, Tampa, FL 33612, USA. jhassell@shctampa.usf.edu
Yamada
Yoshihiko
Y
Arikawa-Hirasawa
Eri
E
D000818:Animals; D001848:Bone Diseases, Developmental / Q000235:genetics; D002356:Cartilage / Q000254:growth & development*; D019812:Heparan Sulfate Proteoglycans / Q000737:chemistry / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D015394:Molecular Structure; D009154:Mutation; D009469:Neuromuscular Junction / Q000502:physiology; D010009:Osteochondrodysplasias / Q000235:genetics
D019812:Heparan Sulfate Proteoglycans; C071927:perlecan