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Homocystinuria (HCU) is an inherited disorder of amino acid metabolism, caused by an enzymatic defect in a metabolic pathway that leads to increased levels of methionine.
1 Cystinuria is a relatively common inherited disorder. 2  The disorder is due to a defect in the transport of amino acids including one called cystine. 3  Cystinuria features too much cystine in the urine.  Cystine is highly insoluble, precipitates out of solution and forms stones in the urine.