PMID: 6113252Jun 1, 1981Paper

Decreased rates of methionine synthesis by methylene tetrahydrofolate reductase-deficient fibroblasts and lymphoblasts

The Journal of Clinical Investigation
G R Boss, R W Erbe

Abstract

Methionine synthesis from homocysteine was measured in intact human fibroblasts and lymphoblasts using a [14C]formate label. Seven fibroblast lines and two lymphoblast lines derived from patients with 5,10-methylene tetrahydrofolate reductase deficiency had rates of methionine synthesis that were from 4 to 43% of normal. When the patients were divided by clinical status into mildly (two patients), moderately (two patients), and severely (three patients) affected, methionine biosynthesis expressed as a percent of control values was 43 and 33%, 11 and 10%, and 7, 6, and 4%, respectively, in fibroblasts. Similar data for the two lymphoblast lines were 36 and 26% for a mildly and moderately affected patient, respectively. These data are to be contrasted with the measurement of residual enzyme activity in cell extracts which agrees less precisely with the clinical status of the patients. In the presence of normal methionine synthetase activity, the rate of synthesis of methionine from homocysteine is a function of the activity of the enzyme 5,10-methylene tetrahydrofolate reductase, and measurement of the methionine biosynthetic capacity of cells deficient in this enzyme accurately reflects the clinical status of the patient from wh...Continue Reading

References

May 1, 1976·Proceedings of the National Academy of Sciences of the United States of America·R M Hoffman, R W Erbe
May 1, 1979·The Journal of Clinical Investigation·D S RosenblattR Baumgartner
Feb 9, 1973·Science·A Yoshida
Sep 1, 1973·Proceedings of the National Academy of Sciences of the United States of America·D KamelyR W Erbe
Apr 1, 1974·Metabolism: Clinical and Experimental·J D Finkelstein
Oct 15, 1973·Biochemical and Biophysical Research Communications·D S Rosenblatt, R W Erbe
Jan 31, 1972·Biochemical and Biophysical Research Communications·S H MuddV E Shih

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Citations

Jul 1, 1985·The Journal of Clinical Investigation·G R Boss
Jun 1, 1983·The Journal of Pediatrics·U WendelE Diekmann
May 24, 2001·The Journal of Biological Chemistry·I O DanishpajoohG R Boss
Jun 1, 1992·Biochemical Medicine and Metabolic Biology·S L SillaotsD S Rosenblatt

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