Abstract
SummaryA one‐year‐old girl with chronic metabolic acidosis was found to have normal renal acidi‐fication. In the urine, however, she excreted a daily amount of 50 mmoles of L‐pyroglutamate (5‐oxo‐˜‐proline). This excretion did not increase with increasing protein intake. Studies of the turnover of L‐pyroglutamate under steady state conditions revealed that 75% of the pyroglutamate synthesized was metabolized by the patient. The level of pyroglutamate degrading enzyme (5‐0times0‐prolinase) in the patient's leucocytes was not decreased, and the enzyme appeared to have normal affinity for its substrates, i.e. pyroglutamate and ATP.
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