Intestinal Transport of Cystine and Cysteine in Man: Evidence for Separate Mechanisms*

Abstract

Cystine and cysteine are transported by energy-dependent, mediated processes in human gut. When either of these amino acids is transported, only cysteine is recovered intracellularly, indicating that cystine is reduced to cysteine after achieving an intracellular location. In contrast to results with cystine, cysteine uptake is not defective in gut from cystinuric patients, nor do lysine and arginine compete with cysteine for transport. It is, therefore, concluded that cystine and cysteine are transported by different mechanisms, and that only the cystine transport mechanism is defective in cystinuria.