l-Cystine Diamides asl-Cystine Crystallization Inhibitors for Cystinuria

Cystinuria: Defective Intestinal Transport of Dibasic Amino Acids and Cystine.

The clinical manifestations of cystinuria are localized to the urinary tract and result from the formation of cystine calculi. The findings of increased excretion of cystine, lysine, arginine, and ornithine in the urine at a time when the plasma levels of these amino acids were normal or low suggested to Dent and Rose (2) that a renal tu-1)ular reabsorptive site, shared by the involved amino ac...

Defective Uptake of Basic Amino Acids and L-cystine by Intestinal Mucosa of Patients with Cystinuria.

Crystal structure of seleno-l-cystine di­hydro­chloride

Numerous crystal structures are available for the dimeric amino acid cystine. In proteins it is formed by oxidation of the -SH thiol groups of two closely spaced cysteine residues, resulting in the formation of a familiar di-sulfide bridge. The title compound [systematic name: (R,R)-1,1'-dicarb-oxy-2,2'-(diselanedi-yl)diethanaminium dichloride], C6H14N2O4Se2 (2+)·2Cl(-), is the first example of...

Cystine Stones

Cystine stones occur in subjects carrying two autosomal mutant alleles resulting in excessive excretion of cystine, ornithine, arginine and lysine – hence the condition cystinuria. About 1:15–25 000 NSW neonates have been found to have cystinuria, with a gene frequency in Australia calculated to be about 1:4000 individuals. Since these studies were published, the mutant alleles have been identi...

Cystine nephrolithiasis

Our understanding of the molecular basis of cystinuria has deepened as the result of the causative genes, SLC3A1 and SLC7A9, being identified. The proteins coded for by these genes form a heterodimer responsible for reabsorption of filtered cystine in the proximal tubule. Failure of this transport system to be targeted to the apical membrane, as in the case of SLC3A1 mutations, or failure of th...