Citrulline metabolism in normal and citrullinemic human lymphocyte lines.

Biochemical Genetics
E B SpectorA D Bloom

Abstract

Citrullinemia is one of the five aminoacidurias associated with the Krebs-Henseleit urea cycle. A long-term lymphocyte line (UM-21) derived from a patient with this disease and nine of ten clones of this line were found to have no activity for the enzyme argininosuccinate synthetase (AS), as demonstrated by their inability to grow in medium in which citrulline had been substituted for arginine, by their inability to incorporate arginine-C14 derived from citrulline-C14 into cellular protein, and by direct enzyme assay. One clone had normal or nearly normal argininosuccinate synthetase activity, as demonstrated by the same criteria. Nutritional "variants" able to grow logarithmically in medium containing citrulline were isolated from UM-21 and three clones. The apparent Kms of AS for citrulline in UM-21, the ten clones, the variant lines, and a normal line were measured and fell into three groups: AS in UM-21 and nine clones had no measurable apparent Km for citrulline; AS in the variant cells had apparent Kms for citrulline of approximately 20 mM; and AS in the normal cell line and one clone had apparent Kms for citrulline of 0.2 mM. The data suggest that the defect in the citrullinemic cell lines is due to a mutation in the str...Continue Reading

References

Feb 1, 1973·Proceedings of the National Academy of Sciences of the United States of America·A L BeaudetC T Caskey
Mar 1, 1974·Proceedings of the National Academy of Sciences of the United States of America·L A ChasinG Urlaub
Nov 1, 1972·Proceedings of the National Academy of Sciences of the United States of America·V L ChanL Siminovitch
Nov 1, 1973·Proceedings of the National Academy of Sciences of the United States of America·J D SharpM R Capecchi
Mar 15, 1974·Experimental Cell Research·L B Jacoby
Jul 1, 1973·Journal of Bacteriology·K N SubramanianR H Davis
Aug 1, 1973·Pediatric Research·E B Spector, A D Bloom
Mar 6, 1967·JAMA : the Journal of the American Medical Association·G E Moore
Jun 1, 1971·Journal of Cellular Physiology·J Morrow
Jul 11, 1970·Nature·K W Choi, A D Bloom
Mar 1, 1967·Proceedings of the National Academy of Sciences of the United States of America·T A Tedesco, W J Mellman

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Citations

Apr 1, 1977·Biochemical Genetics·O LockridgeA D Bloom
Nov 1, 1977·Clinica Chimica Acta; International Journal of Clinical Chemistry·I MatsudaI Akaboshi
Feb 1, 1977·The Journal of Pediatrics·J ThoeneW Nyhan
Dec 11, 2014·Journal of Leukocyte Biology·Tatyana N TarasenkoPeter J McGuire
Mar 10, 1978·Virchows Archiv. A, Pathological Anatomy and Histology·J LeibowitzW Nyhan
May 1, 1978·Life Sciences·J J Wasmuth, C T Caskey

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