Renal clearance of cystathionine in homozygous and heterozygous cystathioninuria, cystinuria, and the normal state

The Journal of Clinical Investigation
G W FRIMPTER, A J Greenberg

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 mumole 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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Citations

Apr 25, 1968·The New England Journal of Medicine·D T Whelan, C R Scriver
Oct 18, 1973·The New England Journal of Medicine·G W Frimpter
May 1, 1969·The American Journal of Medicine·G W Frimpter
Sep 30, 1969·Annals of the New York Academy of Sciences·G W FrimpterR J Andelman
Apr 25, 2014·American Journal of Physiology. Renal Physiology·Noriyuki AkahoshiIsao Ishii
Jun 1, 1989·Journal of Mental Deficiency Research·A HestnesL Gjessing

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