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In vitro sulfate turnover in osteogenesis imperfecta congenita and tarda

✍ Scribed by Delvin, Edgard E. ;Glorieux, Francis H. ;Lopez, Elida ;Opitz, John M. ;Sillence, David

Publisher
John Wiley and Sons
Year
1979
Tongue
English
Weight
475 KB
Volume
4
Category
Article
ISSN
0148-7299

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✦ Synopsis

Sulfate (35 S 0 4 -2 ) uptake was studied in confluent skin fibroblasts from three patients with osteogenesis irnperfecta "congenita," six patients with osteogenesis imperfecta "tarda," three clinically unaffected relatives of an osteogenesis irnperfecta tarda patient, and four controls. Only two of the osteogenesis imperfecta congenita cell strains showed an increased uptake of sulfate, all other cell strains being comparable to the control group. The degradation rate of glycosaminoglycans in mutants as seen by the chase experiments was comparable to that found in the normal control cell strains. Glucose oxidation was normal in the osteogenesis irnperfecta cell strains having an abnormal sulfate uptake. This rules out the possibility of an hypermetabolic state of these cells. These findings do not warrant the use of 35 S 0 4 -2 incorporation in cultured cells as a tool for prenatal diagnosis of osteogenesis irnperfecta.

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