Schnurri-3 is an essential regulator of osteoblast function and adult bone mass.
Skeletal remodelling is a cyclical process where under normal physiological conditions, bone formation occurs at sites where bone resorption has previously taken place. Homeostatic remodelling of the skeleton is mediated by osteoclasts, giant multinucleated cells of haematopoietic origin that are responsible for bone resorption and osteoblasts, which originate from mesenchymal stem cells, and synthesise the matrix constituents on bone-forming surfaces.1 Proliferation, differentiation and bone remodelling activities of these cells involve a complex temporal network of growth factors, signalling proteins and transcription factors. Dysregulation of any one component may disrupt the remodelling process and contribute to the pathogenesis of common skeletal disorders, like osteoporosis and Paget's disease. Rare single gene disorders resulting in elevated bone mass due to osteoclast defects are collectively termed osteopetrosis. Rarer still are single gene disorders, collectively termed osteosclerosis, in which elevated bone mass is due to intrinsically elevated osteoblast activity.2 While we have learned much about the molecular control of skeletal formation and remodelling from these mutations, additional genes that regulate bone mass have yet to be characterised.
Jones
Dallas C
DC
Department of Immunology and Infectious Diseases, Harvard School of Public Health, 651 Huntington Ave, FXB 205, Boston, MA 02115, USA.
Wein
Marc N
MN
Glimcher
Laurie H
LH
D000328:Adult; D000818:Animals; D016723:Bone Remodeling / Q000235:genetics*; D004268:DNA-Binding Proteins / Q000037:antagonists & inhibitors / Q000235:genetics*; D005786:Gene Expression Regulation / Q000235:genetics; D006801:Humans; D059630:Mesenchymal Stem Cells / Q000502:physiology; D051379:Mice; D008875:Middle Aged; D010006:Osteoblasts / Q000502:physiology*; D010012:Osteogenesis / Q000502:physiology; D010024:Osteoporosis / Q000188:drug therapy; D044767:Ubiquitin-Protein Ligases / Q000235:genetics; D016335:Zinc Fingers / Q000235:genetics
D004268:DNA-Binding Proteins; C425103:HIVEP3 protein, human; C471642:WWP1 protein, human; D044767:Ubiquitin-Protein Ligases