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

George W. Frimpter; Abby J. Greenberg

doi:10.1172/JCI105604

^{Department of Medicine, The New York Hospital-Cornell Medical Center, New York, N. Y.}

^{Department of Pediatrics, The New York Hospital-Cornell Medical Center, New York, N. Y.}

†

Established Investigator, American Heart Association.

Address requests for reprints to Dr. George W. Frimpter, The New York Hospital-Cornell Medical Center, 525 E. 68th St., New York, N. Y. 10021.

‡

Postdoctoral trainee in pediatrics (nephrology) supported by U. S. Public Health Service grant 5T1-AM-5455-02.

Submitted for publication July 19, 1966; accepted March 6, 1967.

Supported in part by the following grants from the National Institutes of Health: AM-08404, HE-08736, and FR-47.

Find articles by Frimpter, G. in: JCI | PubMed | Google Scholar

^{Department of Medicine, The New York Hospital-Cornell Medical Center, New York, N. Y.}

^{Department of Pediatrics, The New York Hospital-Cornell Medical Center, New York, N. Y.}

†

Established Investigator, American Heart Association.

Address requests for reprints to Dr. George W. Frimpter, The New York Hospital-Cornell Medical Center, 525 E. 68th St., New York, N. Y. 10021.

‡

Postdoctoral trainee in pediatrics (nephrology) supported by U. S. Public Health Service grant 5T1-AM-5455-02.

Submitted for publication July 19, 1966; accepted March 6, 1967.

Supported in part by the following grants from the National Institutes of Health: AM-08404, HE-08736, and FR-47.

Find articles by Greenberg, A. in: JCI | PubMed | Google Scholar

Published June 1, 1967 - More info

View PDF

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.