Show simple item record

dc.contributor.authorJackson, Mary E.
dc.contributor.authorShalhoub, Victoria
dc.contributor.authorLian, Jane B.
dc.contributor.authorStein, Gary S.
dc.contributor.authorMarks, Sandy C. Jr.
dc.date2022-08-11T08:10:58.000
dc.date.accessioned2022-08-23T17:26:32Z
dc.date.available2022-08-23T17:26:32Z
dc.date.issued1994-07-01
dc.date.submitted2011-01-11
dc.identifier.citationJ Cell Biochem. 1994 Jul;55(3):366-72. <a href="http://dx.doi.org/10.1002/jcb.240550314">Link to article on publisher's site</a>
dc.identifier.issn0730-2312 (Linking)
dc.identifier.doi10.1002/jcb.240550314
dc.identifier.pmid7962169
dc.identifier.urihttp://hdl.handle.net/20.500.14038/49659
dc.description.abstractOsteopetrosis 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.
dc.language.isoen_US
dc.relation<a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&list_uids=7962169&dopt=Abstract">Link to Article in PubMed</a>
dc.relation.urlhttp://dx.doi.org/10.1002/jcb.240550314
dc.subjectAnimals
dc.subjectBone and Bones
dc.subjectCells, Cultured
dc.subjectGene Expression Regulation
dc.subjectOsteoblasts
dc.subjectOsteopetrosis
dc.subjectRats
dc.subjectRats, Mutant Strains
dc.subjectCell Biology
dc.titleAberrant gene expression in cultured mammalian bone cells demonstrates an osteoblast defect in osteopetrosis
dc.typeJournal Article
dc.source.journaltitleJournal of cellular biochemistry
dc.source.volume55
dc.source.issue3
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/stein/88
dc.identifier.contextkey1724130
html.description.abstract<p>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.</p>
dc.identifier.submissionpathstein/88
dc.contributor.departmentDepartment of Cell Biology
dc.source.pages366-72


This item appears in the following Collection(s)

Show simple item record