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| | eMedicine - Citrullinemia : Article by Karl S Roth, MD (Site not responding. Last check: 2007-10-08) |
 | | Under normal circumstances, citrulline is condensed with aspartic acid to form argininosuccinic acid (ASA), which is a reaction mediated by the argininosuccinic acid synthase enzyme. |
 | | Citrulline can be metabolized outside of the liver, and ASA synthase is normally expressed in the brain, kidney, and skin fibroblasts. |
 | | As mentioned, by reaction of citrulline with aspartic acid, a second waste nitrogen molecule is incorporated into the urea cycle; however, this reaction is impaired and results in reduction of the overall capacity of the urea cycle to dispose of ammonia by 50%. |
| www.emedicine.com /ped/topic406.htm (2317 words) |
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