Aberrant gene expression in cultured mammalian bone cells demonstrates an osteoblast defect in osteopetrosis
UMass Chan Affiliations
Department of Cell BiologyDocument Type
Journal ArticlePublication Date
1994-07-01Keywords
AnimalsBone and Bones
Cells, Cultured
Gene Expression Regulation
Osteoblasts
Osteopetrosis
Rats
Rats, Mutant Strains
Cell Biology
Metadata
Show full item recordAbstract
Osteopetrosis is a skeletal condition in which a generalized radioopacity of bone is caused by reduced resorption of bone by osteoclasts. However, it has recently been shown that during skeletal development in several osteopetrotic rat mutations specific aberrations occur in gene expression reflecting the activity of the bone forming cells, osteoblasts, and the development of tissue organization. To evaluate their pathogenetic significance, progressive osteoblast differentiation was studied in vitro. Primary cultures of normal osteoblasts undergo a sequential expression of cell growth and tissue-related genes associated with development of skeletal tissue. We report that osteoblast cultures can be established from one of these mutants, toothless; that these cells in vitro exhibit similar aberrations in gene expression during cell proliferation and extracellular matrix formation and mineralization observed in vivo; and that an accelerated maturation sequence by mutant osteoblasts mimics the characteristic skeletal sclerosis of this disease. These data are the first direct evidence for an intrinsic osteoblast defect in osteopetrosis and establish an in vitro model for the study of heritable skeletal disorders.Source
J Cell Biochem. 1994 Jul;55(3):366-72. Link to article on publisher's siteDOI
10.1002/jcb.240550314Permanent Link to this Item
http://hdl.handle.net/20.500.14038/49659PubMed ID
7962169Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1002/jcb.240550314