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ENTRY	nt06010
Name	Urea cycle
Category	Pathway view; Amino acid metabolism
Pathway	hsa00220 Arginine biosynthesis
Module	hsa_M00029 Urea cycle
Disease	H01398 Urea cycle disorders
Display	drug-target relation   disease type

D1  |                     N00593    NH3 − CPS1 ⇉ OTC ⇉ ASS1 ⇉ ASL ⇉ ARG1/2 → Urea+Ornithine     CPS1 deficiency     CPS1*     OTC deficiency         OTC*     ASS deficiency             ASS1*     ASL deficiency                 ASL*     ARG1 deficiency                     ARG1*               D1  | N00599    Glu − NAGS → NAG = CPS1               D1  | N00600    NAGS deficiency Glu − NAGS* ⌿ NAG ⌿ CPS1

		Disease name	Disease category
CPS1 deficiency	H00164	Carbamoyl phosphate synthetase I deficiency	Inherited metabolic disorder
OTC deficiency	H00187	Ornithine transcarbamylase deficiency	Inherited metabolic disorder
ASS deficiency	H00185	Citrullinemia	Inherited metabolic disorder
ASL deficiency	H01028	Argininosuccinic aciduria	Inherited metabolic disorder
ARG1 deficiency	H00186	Hyperargininemia	Inherited metabolic disorder
NAGS deficiency	H01032	N-acetylglutamate synthase deficiency	Inherited metabolic disorder

		Drug name
D1	D07130	Carglumic acid (JAN/USAN/INN)