{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,1,30]],"date-time":"2024-01-30T20:46:49Z","timestamp":1706647609857},"reference-count":28,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,2,19]],"date-time":"2004-02-19T00:00:00Z","timestamp":1077148800000},"content-version":"vor","delay-in-days":3277,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Cellular Biochemistry"],"published-print":{"date-parts":[[1995,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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\u2010derived cells affected with NF1 were tested in culture for specific alkaline phosphatase (ALP) activity and cell\u2010mediated 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 + \u03b2\u2010glycerophosphate (\u03b2GP) (Dex medium) coordinately with a decrease in cell proliferation. 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 \u03b2GP resulted in mineralization by NF1 cells but less than in other cells. In addition, NF1 cells responded to dexamethasone by increasing the \u03b2GP\u2010induced mineralization, as opposed to cells from other embryonic bones, which either did not respond or have even decreased mineralization under dexamethasone. Upon cis\u2010hydroxyproline 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\u2010derived 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\u2010treated cells, at days 0\u201311 has inhibited day 21 mineralization in all tested cultures in spite of the increase in day 11\u2010specific ALP activity. Both malonate and levamisole did not distinguish NF1 cells from the osteogenic phenotype of other cells. Essentially embryonic bone\u2010derived cells from 12 weeks gestation, cultured in the absence of \u03b2GP, retained their mineralization capacity, which does not increase under dexamethasone, as distinguished from NF1 cells which require \u03b2GP for mineralization and positively respond to dexamethasone. Therefore, bone\u2010derived NF1 cells may be useful for studying the regulation of the mineralization process.<\/jats:p>","DOI":"10.1002\/jcb.240570318","type":"journal-article","created":{"date-parts":[[2005,5,28]],"date-time":"2005-05-28T22:07:11Z","timestamp":1117318031000},"page":"530-542","source":"Crossref","is-referenced-by-count":12,"title":["Analysis of cell\u2010mediated mineralization in culture of bone\u2010derived embryonic cells with neurofibromatosis"],"prefix":"10.1002","volume":"57","author":[{"suffix":"Dr.","given":"B. 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