The aetiology of canine cystinuria.

A mouse model for cystinuria type I.

Cystinuria, one of the most common inborn errors of metabolism in humans, accounts for 1-2% of all cases of renal lithiasis. It is caused by defects in the heterodimeric transporter system rBAT/b0,+AT, which lead to reduced reabsorption of cystine and dibasic amino acids through the epithelial cells of the renal tubules and the intestine. In an N-ethyl-N-nitrosourea mutagenesis screen for reces...

The chromatographie studies of a case of cystinuria in Iran

Linköping University Medical Dissertations

Cystinuria is defined as an inherited disorder characterised by increased urinary excretion of cystine and the dibasic amino acids arginine, lysine and ornithine. The only clinical manifestation of cystinuria is renal cystine stone formation due to the low solubility of cystine in the urine. Cystinuria can be attributed to mutations in the SLC3A1 and SLC7A9 genes in the majority of all cases an...

THE CAPTIVE MANED WOLF (Chrysocyon brachyurus): NUTRITIONAL CONSIDERATIONS WITH EMPHASIS ON MANAGEMENT OF CYSTINURIA

Title of Thesis: THE CAPTIVE MANED WOLF (Chrysocyon brachyurus): NUTRITIONAL CONSIDERATIONS WITH EMPHASIS ON MANAGEMENT OF CYSTINURIA Degree Candidate: Sara E. Childs-Sanford Master of Science, 2005 Thesis directed by: Dr. Roselina Angel Department of Animal and Avian Sciences The intent of this project was to investigate options for improvement of a commercially manufactured prescription diet ...

Apical heterodimeric cystine and cationic amino acid transporter expressed in MDCK cells.

The luminal uptake of L-cystine and cationic amino acids by (re)absorptive epithelia, as found in the small intestine and the proximal kidney tubule, is mediated by the transport system b(0,+), which is defective in cystinuria. Expression studies in Xenopus laevis oocytes and other nonepithelial cells as well as genetic studies on cystinuria patients have demonstrated that two gene products, th...