Document Detail

Analysis of cell-mediated mineralization in culture of bone-derived embryonic cells with neurofibromatosis.
MedLine Citation:
PMID:  7768987     Owner:  NLM     Status:  MEDLINE    
von Recklinghausen neurofibromatosis (NF1) is an autosomal dominant genetic disorder associated with congenital pseudoarthrosis and with short stature. To examine whether the NF1 phenotype includes functional osteogenic defects, embryonic bone-derived cells affected with NF1 were tested in culture for specific alkaline phosphatase (ALP) activity and cell-mediated mineralization and compared with other embryonic bone derived cells. NF1 showed a relatively higher specific ALP activity, which has further increased in response to dexamethasone + beta-glycerophosphate (beta GP) (Dex medium) coordinately with a decrease in cell proliferation. In In the control group, two samples showed increased ALP activity, one showed decreased activity and the forth one did not show any change in ALP. NF1 cells were distinguished from other cells regarding day 21 mineralization, they did not mineralize when cultured with ascorbate alone in the absence of Dex medium, whereas control cells did mineralize. Adding beta GP resulted in mineralization by NF1 cells but less than in other cells. In addition, NF1 cells responded to dexamethasone by increasing the beta GP-induced mineralization, as opposed to cells from other embryonic bones, which either did not respond or have even decreased mineralization under dexamethasone. Upon cis-hydroxyproline exposure, Dex medium has also distinguished NF1 cell ALP activity from that of other cell origins. Inhibition of respiratory complex II by malonate showed that most embryonic bone-derived cells of 12 weeks gestation are malonate resistant; thus, malonate selection was ineffective. This is in contrast to rat marrow stromal cells previously shown to undergo mineralizing cell enrichment in response to malonate. Exposure to levamisole, of Dex-treated cells, at days 0-11 has inhibited day 21 mineralization in all tested cultures in spite of the increase in day 11-specific ALP activity. Both malonate and levamisole did not distinguish NF1 cells from the osteogenic phenotype of other cells. Essentially embryonic bone-derived cells from 12 weeks gestation, cultured in the absence of beta GP, retained their mineralization capacity, which does not increase under dexamethasone, as distinguished from NF1 cells which require beta GP for mineralization and positively respond to dexamethasone. Therefore, bone-derived NF1 cells may be useful for studying the regulation of the mineralization process.
B Y Klein; N Rojansky; I Gal; Z Shlomai; M Liebergall; H Ben-Bassat
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of cellular biochemistry     Volume:  57     ISSN:  0730-2312     ISO Abbreviation:  J. Cell. Biochem.     Publication Date:  1995 Mar 
Date Detail:
Created Date:  1995-07-06     Completed Date:  1995-07-06     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8205768     Medline TA:  J Cell Biochem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  530-42     Citation Subset:  IM    
Laboratory of Experimental Surgery, Hadassah Hospital, Ein-Kerem, Jerusalem, Israel.
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MeSH Terms
Alkaline Phosphatase / metabolism*
Cell Division / drug effects
Cells, Cultured
Embryo, Mammalian / cytology,  metabolism
Embryonic and Fetal Development / physiology
Genes, Dominant*
Hydroxyproline / pharmacology
Levamisole / pharmacology
Malonates / pharmacology
Neurofibromatosis 1 / genetics*,  metabolism,  pathology
Osteogenesis / physiology*
Sequence Homology, Amino Acid
Reg. No./Substance:
0/Malonates; 141-82-2/malonic acid; 14769-73-4/Levamisole; 51-35-4/Hydroxyproline; EC Phosphatase

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