In Cystinuria, all of the following amino acids are excreted in urine, except:-
Cystinuria (cystine-lysinuria): It is one of the most common inherited diseases (it is Autosomal Recessive). Increased excretion of amino acids in urine:- Cystine (25-40 times normal)(most common) Lysine Arginine Ornithine In cystinuria,Specific carrier system in kidney tubules for the reabsorption of amino acids (cysteine, ornithine, arginine and lysine (remember COAL to recall) become defective Defect in dibasic amino acid transpoer Most common inborn error of amino acid transpo Formation of cystine stones in kidney and urinary tract Cyanide nitroprusside test laboratory test Treatment:- -Restricted ingestion of dietary cystine -high intake of fluids -Chelating agent Penicillamine (forms complex with cysteine) ADDITIONAL INFORMATION ON CYSTINURIA Pa of Garrod's tetrad
Cystine
Ornithine
Leucine
Arginine
NEET 2018