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Guanidinosuccinic Aciduria: an Alternate Route of Ammonia Detoxification in Uremia.

Burton D. Cohen, M.D., F.A.C.P.; Israel Stein, M.D.; and John E. Bonas, M.D.
Ann Intern Med. 1966;64(5):1171. doi:10.7326/0003-4819-64-5-1171_1
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In 1963, we reported the isolation of an unknown substance from the urine of uremic subjects. The following year, the material was identified as guanidinosuccinic acid. This represents the first reported instance in which this compound has been isolated from any biologic system. The present report amplifies these observations and introduces data concerning its metabolic source and its role in the uremic syndrome.

Isolation of the material, along with creatinine, arginine, and guanidinoacetic acid, was performed on urine from uremic patients and compared with that from control subjects. Quantitation and identification were accomplished by means of both column chromatography and


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