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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Figure 1.

Figure 1.

Metabolic fates of arginine

Arginine is derived from dietary sources, protein catabolism, or endogenous synthesis. The arginine-citrulline cycle is responsible for regeneration of arginine in various tissues. Arginine serves as the precursor for many biologically important molecules; a decrease in arginine may result in decreased production of compounds for which it serves as a precursor.

GATM = glycine amidinotransferase

Arg1 = arginase 1

ADC = arginine decarboxylase

NOS = nitric oxide synthase

NO = nitric oxide

OTC = ornithine transcarbamoylase

ASS = argininosuccinate synthase

ASA = argininosuccinic acid

ASL = argininosuccinate lyase

GABA = γ-amino butyric acid

From: Argininosuccinate Lyase Deficiency

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