L-Glutamate

N-acetyl-L-Glutamate

N-acetyl-L-Glutamyl-5-phosphate

N-acetyl-L-Glutamyl-5-semialdehyde

N-acetyl-L-Ornithine

L-Ornithine

L-Citrulline

L-argininosuccinate

L-arginine

carbamoylphosphate

acetate

L-Glutamate

acetyl-CoA argA

argB

argC

argD

argE

argF

argG

argH

1

2

3

4

5

6

7

8

5'

N-acetylglutamate synthase

acetylornithinase

ornithine carbamoyltransferase

argininosuccinase

NAGS

OTC

AOargJornithine acetyltransferase

OAT2.3.1.35 3.5.1.16

2.1.3.3

2.3.1.1

2.7.2.8

4.3.2.1

1.2.1.38

N-acetyl-gamma-glutamyl-phosphate

reductase

N-acetylglutamate kinase NAGK

acetylornithine/succinyldiaminopime

late aminotransferase

ACOAT 2.6.1.11 2.6.1.17

argininosuccinate synthase 6.3.4.5