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Perlecan, A Multi-Functional, Cell-Instructive, Matrix-Stabilizing Proteoglycan With Roles in Tissue Development Has Relevance to Connective Tissue Repair and Regeneration.
Front Cell Dev Biol
April 2022
Graduate School of Biomedical Engineering, University of New South Wales, Sydney, NSW, Australia.
This review highlights the multifunctional properties of perlecan (HSPG2) and its potential roles in repair biology. Perlecan is ubiquitous, occurring in vascular, cartilaginous, adipose, lymphoreticular, bone and bone marrow stroma and in neural tissues. Perlecan has roles in angiogenesis, tissue development and extracellular matrix stabilization in mature weight bearing and tensional tissues.
View Article and Find Full Text PDFBone maturation in girls with Turner's syndrome.
Eur J Endocrinol
January 1998
Department of Pediatrics, Bnai Zion Medical Center, Haifa, Israel.
The mechanism of growth retardation in Turner's syndrome has not been resolved. It is often referred to as a bone dysplasia, although endocrine derangement has not been ruled out. The present study was undertaken to evaluate the maturation of individual bones of the hand and wrist in girls with Turner's syndrome and thereby obtain information which may aid in elaborating the possible mechanism of the growth retardation in girls with Turner's syndrome.
View Article and Find Full Text PDFLinkage studies of a Missouri kindred with autosomal dominant spondyloepimetaphyseal dysplasia (SEMD) indicate genetic heterogeneity.
J Bone Miner Res
August 1997
MRC Molecular Endocrinology Group, MRC Clinical Sciences Centre, Royal Postgraduate Medical School, London, United Kingdom.
A four-generation kindred (14 affected and 10 unaffected members) from Missouri, U.S.A.
View Article and Find Full Text PDFHypochrondroplasia is an inherited skeletal dysplasia that resembles achondroplasia in mild degree. Radiographic manifestations encountered in 12 affected individuals in South Africa include slight shortening of all segments of the tubular bones, moderate caudal diminution of the lumbar interpedicular distances, increased lumbar lordosis with sacral tilt and distal prolongation of the fibula. Hypochondroplasia can be distinguished from other osteochondrodystrophies such as achondroplasia, pseudoachondroplasia and metaphyseal chondroplasia by the recognition of its clinical and radiographic manifestations.
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