JAWS coordinates chondrogenesis and synovial joint positioning
Open Access
- 1 July 2008
- journal article
- Published by The Company of Biologists in Development
- Vol. 135 (13), 2215-2220
- https://doi.org/10.1242/dev.019950
Abstract
Properly positioned synovial joints are crucial to coordinated skeletal movement. Despite their importance for skeletal development and function, the molecular mechanisms that underlie joint positioning are not well understood. We show that mice carrying an insertional mutation in a previously uncharacterized gene, which we have named Jaws (joints abnormal with splitting), die perinatally with striking skeletal defects, including ectopic interphalangeal joints. These ectopic joints develop along the longitudinal axis and persist at birth, suggesting that JAWS is uniquely required for the orientation and consequent positioning of interphalangeal joints within the endochondral skeleton. Jaws mutant mice also exhibit severe chondrodysplasia characterized by delayed and disorganized maturation of growth plate chondrocytes, together with impaired chondroitin sulfation and abnormal metabolism of the chondroitin sulfate proteoglycan aggrecan. Our findings identify JAWS as a key regulator of chondrogenesis and synovial joint positioning required for the restriction of joint formation to discrete stereotyped locations in the embryonic skeleton.Keywords
This publication has 30 references indexed in Scilit:
- Hif-1α regulates differentiation of limb bud mesenchyme and joint developmentThe Journal of cell biology, 2007
- Sulf1 expression pattern and its role in cartilage and joint developmentDevelopmental Dynamics, 2006
- Wnt/β-catenin signaling interacts differentially with Ihh signaling in controlling endochondral bone and synovial joint formationDevelopment, 2006
- Mechanisms of synovial joint and articular cartilage formation: Recent advances, but many lingering mysteriesBirth Defects Research Part C: Embryo Today: Reviews, 2005
- Maintenance of chondroitin sulfation balance by chondroitin-4-sulfotransferase 1 is required for chondrocyte development and growth factor signaling during cartilage morphogenesisDevelopment, 2005
- Canonical Wnt Signaling in Differentiated Osteoblasts Controls Osteoclast DifferentiationDevelopmental Cell, 2005
- Coordination of chondrocyte differentiation and joint formation byα5β1 integrin in the developing appendicular skeletonDevelopment, 2004
- Developmental regulation of the growth plateNature, 2003
- The Thrombospondin Motif of Aggrecanase-1 (ADAMTS-4) Is Critical for Aggrecan Substrate Recognition and CleavagePublished by Elsevier BV ,2000
- Development of the metatarsophalangeal joint of the chick embryo: Morphological, ultrastructural and histochemical studiesJournal of Anatomy, 1977