In vitro sulfate turnover in osteogenesis imperfacta congenita and tarda
Journal Article
·
· Am. J. Med. Genet.; ()
Sulfate (/sup 35/SO/sub 4//sup -2/) uptake was studied in confluent skin fibroblasts from three patients with osteogenesis imperfecta congenita, six patients with osteogenesis imperfecta tarda, three clinically unaffected relatives of an osteogenesis imperfecta 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 glycosaminolgycans 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 imperfecta 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 /sup 35/SO/sub 4//sup -2/ incorporation in cultured cells as a tool for prenatal diagnosis of osteogenesis imperfecta.
- Research Organization:
- Shriners Hospital for Crippled Children, Montreal, Canada
- OSTI ID:
- 5891788
- Journal Information:
- Am. J. Med. Genet.; (), Journal Name: Am. J. Med. Genet.; () Vol. 4:4
- Country of Publication:
- United States
- Language:
- English
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Related Subjects
550501 -- Metabolism-- Tracer Techniques
560306* -- Chemicals Metabolism & Toxicology-- Man-- (-1987)
59 BASIC BIOLOGICAL SCIENCES
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ANIMAL TISSUES
ANIMALS
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BODY
BONE TISSUES
CONNECTIVE TISSUE
DAYS LIVING RADIOISOTOPES
EVEN-ODD NUCLEI
ISOTOPES
LIGHT NUCLEI
MAMMALS
MAN
METABOLISM
NUCLEI
ORGANS
OXYGEN COMPOUNDS
PRIMATES
RADIOISOTOPES
SKIN
SULFATES
SULFUR 35
SULFUR COMPOUNDS
SULFUR ISOTOPES
TERATOGENESIS
TISSUES
UPTAKE
VERTEBRATES
560306* -- Chemicals Metabolism & Toxicology-- Man-- (-1987)
59 BASIC BIOLOGICAL SCIENCES
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ANIMAL TISSUES
ANIMALS
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BODY
BONE TISSUES
CONNECTIVE TISSUE
DAYS LIVING RADIOISOTOPES
EVEN-ODD NUCLEI
ISOTOPES
LIGHT NUCLEI
MAMMALS
MAN
METABOLISM
NUCLEI
ORGANS
OXYGEN COMPOUNDS
PRIMATES
RADIOISOTOPES
SKIN
SULFATES
SULFUR 35
SULFUR COMPOUNDS
SULFUR ISOTOPES
TERATOGENESIS
TISSUES
UPTAKE
VERTEBRATES