| Long-term extracellular matrix metabolism by cultured human osteogenesis imperfecta osteoblasts. | |
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MedLine Citation:
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PMID: 8725177 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Osteopenia due to deficient extracellular matrix synthesis is a hallmark of osteogenesis imperfecta (OI), Previous studies carried out within 72 h of osteoblast subculture, at an early stage of matrix synthesis, indicated that for osteoblasts derived from human OI patients the total amounts of collagen, osteonectin, and three proteoglycans were significantly reduced, while total amounts of thrombospondin, fibronectin, and matrix hyaluronan were elevated compared with age-matched controls. The current study was undertaken to follow OI osteoblast matrix metabolism as that matrix is synthesized, deposited, and matured. Steady-state metabolic radiolabeling was used to follow the metabolism of collagen, hyaluronan, and total proteoglycan by OI and normal osteoblasts for up to 5 weeks. Trabecular osteoblasts from non-OI controls showed an increase in total and matrix-associated collagen synthesis during the first and second week, reaching steady-state levels by week 4. In contrast, cultured OI osteoblasts did not increase either the total (medium + matrix-associated) or matrix-associated collagen during the entire 5-week period. Proteoglycan synthesis exhibited a pattern similar to that for collagen. OI-derived proteoglycans differed from controls in that levels in OI cultures did not reflect the normal time-dependent increase in total proteoglycan and proteoglycan matrix deposition. OI osteoblast hyaluronan synthesis was increased when compared with age-matched controls during 4 weeks of culture. In contrast, the ratios of calcium to phosphorus solublized from control and the OI extracellular matrix were not statistically different. Thus, with respect to the synthesis of collagen, proteoglycans, and hyaluronan, OI osteoblasts fail to parallel controls in depositing and elaborating extracellular matrix during 35 days in culture. |
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Authors:
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N S Fedarko; P D Sponseller; J R Shapiro |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research Volume: 11 ISSN: 0884-0431 ISO Abbreviation: J. Bone Miner. Res. Publication Date: 1996 Jun |
Date Detail:
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Created Date: 1996-12-23 Completed Date: 1996-12-23 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 8610640 Medline TA: J Bone Miner Res Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 800-5 Citation Subset: IM |
Affiliation:
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Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Age Factors Calcium / analysis Cells, Cultured Child Collagen / biosynthesis Extracellular Matrix / metabolism* Humans Hyaluronic Acid / biosynthesis Osteoblasts / metabolism* Osteogenesis Imperfecta / metabolism* Phosphorus / analysis Proteoglycans / biosynthesis Time Factors |
| Grant Support | |
ID/Acronym/Agency:
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AR 39870/AR/NIAMS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Proteoglycans; 7440-70-2/Calcium; 7723-14-0/Phosphorus; 9004-61-9/Hyaluronic Acid; 9007-34-5/Collagen |
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