Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice
Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice. / Xu, T; Bianco, P; Fisher, L W; Longenecker, G; Smith, E; Goldstein, S; Bonadio, J; Boskey, A; Heegaard, Anne-Marie; Sommer, B; Satomura, K; Dominguez, P; Zhao, C; Kulkarni, A B; Robey, P G; Young, M F.
I: Nature Genetics, Bind 20, Nr. 1, 1998, s. 78-82.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Targeted disruption of the biglycan gene leads to an osteoporosis-like phenotype in mice
AU - Xu, T
AU - Bianco, P
AU - Fisher, L W
AU - Longenecker, G
AU - Smith, E
AU - Goldstein, S
AU - Bonadio, J
AU - Boskey, A
AU - Heegaard, Anne-Marie
AU - Sommer, B
AU - Satomura, K
AU - Dominguez, P
AU - Zhao, C
AU - Kulkarni, A B
AU - Robey, P G
AU - Young, M F
PY - 1998
Y1 - 1998
N2 - The resilience and strength of bone is due to the orderly mineralization of a specialized extracellular matrix (ECM) composed of type I collagen (90%) and a host of non-collagenous proteins that are, in general, also found in other tissues. Biglycan (encoded by the gene Bgn) is an ECM proteoglycan that is enriched in bone and other non-skeletal connective tissues. In vitro studies indicate that Bgn may function in connective tissue metabolism by binding to collagen fibrils and TGF-beta (refs 5,6), and may promote neuronal survival. To study the role of Bgn in vivo, we generated Bgn-deficient mice. Although apparently normal at birth, these mice display a phenotype characterized by a reduced growth rate and decreased bone mass due to the absence of Bgn. To our knowledge, this is the first report in which deficiency of a non-collagenous ECM protein leads to a skeletal phenotype that is marked by low bone mass that becomes more obvious with age. These mice may serve as an animal model to study the role of ECM proteins in osteoporosis.
AB - The resilience and strength of bone is due to the orderly mineralization of a specialized extracellular matrix (ECM) composed of type I collagen (90%) and a host of non-collagenous proteins that are, in general, also found in other tissues. Biglycan (encoded by the gene Bgn) is an ECM proteoglycan that is enriched in bone and other non-skeletal connective tissues. In vitro studies indicate that Bgn may function in connective tissue metabolism by binding to collagen fibrils and TGF-beta (refs 5,6), and may promote neuronal survival. To study the role of Bgn in vivo, we generated Bgn-deficient mice. Although apparently normal at birth, these mice display a phenotype characterized by a reduced growth rate and decreased bone mass due to the absence of Bgn. To our knowledge, this is the first report in which deficiency of a non-collagenous ECM protein leads to a skeletal phenotype that is marked by low bone mass that becomes more obvious with age. These mice may serve as an animal model to study the role of ECM proteins in osteoporosis.
KW - Age Factors
KW - Animals
KW - Biglycan
KW - Bone Density
KW - Bone Development
KW - Bone and Bones
KW - Extracellular Matrix Proteins
KW - Female
KW - Femur
KW - Gene Expression Regulation, Developmental
KW - Male
KW - Mice
KW - Mice, Inbred C57BL
KW - Mice, Knockout
KW - Osteoporosis
KW - Phenotype
KW - Proteoglycans
KW - Tibia
U2 - 10.1038/1746
DO - 10.1038/1746
M3 - Journal article
C2 - 9731537
VL - 20
SP - 78
EP - 82
JO - Nature Genetics
JF - Nature Genetics
SN - 1061-4036
IS - 1
ER -
ID: 38426650