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. 1967 Jun;46(6):975–982. doi: 10.1172/JCI105604

Renal Clearance of Cystathionine in Homozygous and Heterozygous Cystathioninuria, Cystinuria, and the Normal State*

George W Frimpter 1,2,, Abby J Greenberg 1,2,
PMCID: PMC297102  PMID: 6026103

Abstract

Cystathionine is more readily cleared from the plasma than other amino acids. This is because the amino acid has a very low tubular maximum (Tm), in the order of 1 μmole per minute per 1.73 square meters body surface area (BSA). No essential differences in the reabsorption of cystathionine were observed in four normal subjects, two patients with homozygous cystathioninuria, one patient with heterozygous cystathioninuria, and one patient with cystinuria.

Apparent net tubular secretion of cystathionine was demonstrated in a child with homozygous cystathioninuria only after an intravenously administered load of lysine.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ASATOOR A. M., LACEY B. W., LONDON D. R., MILNE M. D. Aminoacid metabolism in cystinuria. Clin Sci. 1962 Oct;23:285–304. [PubMed] [Google Scholar]
  2. BRENTON D. P., CUSWORTH D. C., GAULL G. E. HOMOCYSTINURIA. BIOCHEMICAL STUDIES OF TISSUES INCLUDING A COMPARISON WITH CYSTATHIONINURIA. Pediatrics. 1965 Jan;35:50–56. [PubMed] [Google Scholar]
  3. Berlow S. Studies in cystathioninemia. Am J Dis Child. 1966 Aug;112(2):135–142. doi: 10.1001/archpedi.1966.02090110079007. [DOI] [PubMed] [Google Scholar]
  4. CRAWHALL J. C., SCOWEN E. F., WATTS R. W. Effect of penicillamine on cystinuria. Br Med J. 1963 Mar 2;1(5330):588–590. doi: 10.1136/bmj.1.5330.588. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. DENT C. E., ROSE G. A. Aminoacid metabolism in cystinuria. Q J Med. 1951 Jul;20(79):205–219. [PubMed] [Google Scholar]
  6. DOOLAN P. D., HARPER H. A., HUTCHIN M. E., ALPEN E. L. Renal clearance of lysine in cystinuria: pathogenesis and management of this abnormality. Am J Med. 1957 Sep;23(3):416–425. doi: 10.1016/0002-9343(57)90321-2. [DOI] [PubMed] [Google Scholar]
  7. FRIMPTER G. W. CYSTINURIA: METABOLISM OF THE DISULFIDE OF CYSTEINE AND HOMOCYSTEINE. J Clin Invest. 1963 Dec;42:1956–1964. doi: 10.1172/JCI104882. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. FRIMPTER G. W., HAYMOVITZ A., HORWITH M. Cystathioninura. N Engl J Med. 1963 Feb 14;268:333–339. doi: 10.1056/NEJM196302142680701. [DOI] [PubMed] [Google Scholar]
  9. FRIMPTER G. W., HORWITH M., FURTH E., FELLOWS R. E., THOMPSON D. D. Inulin and endogenous amino acid renal clearances in cystinuria: evidence for tubular secretion. J Clin Invest. 1962 Feb;41:281–288. doi: 10.1172/JCI104481. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. GJESSING L. R. STUDIES OF FUNCTIONAL NEURAL TUMORS. II. CYSTATHIONINURIA. Scand J Clin Lab Invest. 1963;15:474–478. [PubMed] [Google Scholar]
  11. GORESKY C. A., WATANABE H., JOHNS D. G. THE RENAL EXCRETION OF FOLIC ACID. J Clin Invest. 1963 Dec;42:1841–1849. doi: 10.1172/JCI104868. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. HARRIS H., PENROSE L. S., THOMAS D. H. Cystathioniuria. Ann Hum Genet. 1959 Dec;23:442–453. doi: 10.1111/j.1469-1809.1959.tb01485.x. [DOI] [PubMed] [Google Scholar]
  13. Mongeau J. G., Hilgartner M., Worthen H. G., Frimpter G. W. Cystatbioninuria: study of an infant with normal mentality, thrombocytopenia, and renal calculi. J Pediatr. 1966 Dec;69(6):1113–1120. doi: 10.1016/s0022-3476(66)80303-7. [DOI] [PubMed] [Google Scholar]
  14. ROBSON E. B., ROSE G. A. The effect of intravenous lysine on the renal clearances of cystine, arginine and ornithine in normal subjects, in patients with cystinuria and Fanconi syndrome and in their relatives. Clin Sci. 1957 Feb;16(1):75–93. [PubMed] [Google Scholar]
  15. SCHREINER G. E. Determination of inulin by means of resorcinol. Proc Soc Exp Biol Med. 1950 May;74(1):117–120. doi: 10.3181/00379727-74-17827. [DOI] [PubMed] [Google Scholar]
  16. STEIN W. H., MOORE S. The free amino acids of human blood plasma. J Biol Chem. 1954 Dec;211(2):915–926. [PubMed] [Google Scholar]
  17. Schwartzman L., Blair A., Segal S. A common renal transport system for lysine, ornithine, arginine and cysteine. Biochem Biophys Res Commun. 1966 Apr 19;23(2):220–226. doi: 10.1016/0006-291x(66)90531-6. [DOI] [PubMed] [Google Scholar]
  18. WALSER M., DAVIDSON D. G., ORLOFF J. The renal clearance of alkali-stable inulin. J Clin Invest. 1955 Oct;34(10):1520–1523. doi: 10.1172/JCI103204. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. WEBBER W. A., BROWN J. L., PITTS R. F. Interactions of amino acids in renal tubular transport. Am J Physiol. 1961 Feb;200:380–386. doi: 10.1152/ajplegacy.1961.200.2.380. [DOI] [PubMed] [Google Scholar]

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