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When arginine concentrations become elevated, the ornithine contributed from the urea cycle plus that from 1 -pyrroline-5-carboxylate inhibit the aminotransferase reaction, with accumulation of 1 -pyrroline-5-carboxylate as a result

doi:10.1016/j.rmed.2009.06.016

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Adherence to the treatment regimen, which involves regular injections, is key to maintaining progress