title: The role of the SIBLING, Bone Sialoprotein in skeletal biology - Contribution of mouse experimental genetics.
abstract: Bone Sialoprotein (BSP) is a member of the "Small Integrin-Binding Ligand N-linked Glycoproteins" (SIBLING) extracellular matrix protein family of mineralized tissues. BSP has been less studied than other SIBLING proteins such as Osteopontin (OPN), which is coexpressed with it in several skeletal cell types. Here we review the contribution of genetically engineered mice (BSP gene knockout and overexpression) to the understanding of the role of BSP in the bone organ. The studies made so far highlight the role of BSP in skeletal mineralization, as well as its importance for proper osteoblast and osteoclast differentiation and activity, most prominently in primary/repair bone. The absence of BSP also affects the local environment of the bone tissue, in particular hematopoiesis and vascularization. Interestingly, lack of BSP induces an overexpression of OPN, and the cognate protein could be responsible for some aspects of the BSP gene knockout skeletal phenotype, while replacing BSP for some of its functions. Such interplay between the partly overlapping functions of SIBLING proteins, as well as the network of cross-regulations in which they are involved should now be the focus of further work.
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Wafa
authors.initials: W
authors.lastname: Bouleftour
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Laura
authors.initials: L
authors.lastname: Juignet
authors.affiliation: Department of Haematology, University of Cambridge and NHS Blood and Transplant, Cambridge, UK.
authors.forename: Guenaelle
authors.initials: G
authors.lastname: Bouet
authors.affiliation: Universidade Federal de São Paulo, Santos, Brazil.
authors.forename: Renata Neves
authors.initials: RN
authors.lastname: Granito
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Vanden-Bossche
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Norbert
authors.initials: N
authors.lastname: Laroche
authors.affiliation: Department of Molecular Genetics, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Jane E
authors.initials: JE
authors.lastname: Aubin
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Marie-Hélène
authors.initials: MH
authors.lastname: Lafage-Proust
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Vico
authors.affiliation: Université de Lyon - Université Jean Monnet, INSERM U1059-LBTO/IFRESIS, Faculté de Médecine, 10 Chemin de la Marandière, St Priest en Jarez F42270, France. Electronic address: luc.malaval@univ-st-etienne.fr.
authors.forename: Luc
authors.initials: L
authors.lastname: Malaval
mesh_terms: D000818:Animals; D001842:Bone and Bones / Q000502:physiology*; D002113:Calcification, Physiologic; D002454:Cell Differentiation; D006801:Humans; D058574:Integrin-Binding Sialoprotein / Q000235:genetics* / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D010006:Osteoblasts / Q000166:cytology; D010010:Osteoclasts / Q000166:cytology; D053495:Osteopontin / Q000378:metabolism; D014070:Tooth / Q000502:physiology*
chemical_list: C546872:Ibsp protein, mouse; D058574:Integrin-Binding Sialoprotein; C506896:Spp1 protein, mouse; D053495:Osteopontin
keywords: Bone Sialoprotein; Bone repair; Knockout mice; Modeling; Osteopontin; PTH; Remodeling; SIBLING proteins; Transgenic mice