title: Role of perlecan in skeletal development and diseases.
abstract: 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.
authors.affiliation: The Center for Research in Skeletal Development and Pediatric Orthopaedics, Shriners Hospitals for Children, Tampa, FL 33612, USA. jhassell@shctampa.usf.edu
authors.forename: John
authors.initials: J
authors.lastname: Hassell
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Yamada
authors.forename: Eri
authors.initials: E
authors.lastname: Arikawa-Hirasawa
mesh_terms: 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
chemical_list: D019812:Heparan Sulfate Proteoglycans; C071927:perlecan