N-Acetylglutamate synthase
- N-Acetylglutamate synthase
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N-acetylglutamate synthase is an enzyme that catalyses the production of N-acetylglutamate from acetyl-CoA and glutamate.
This enzyme is important in bacteria and plants for the synthesis of arginine, an amino acid in the pathway.
This enzyme is also important for mammals, because it produces the regulator of urea cycle N-acetylglutamate, which activates carbamoyl phosphate synthetase I, which catalyses the initial reactions of urea cycle.
Clinical significance
Mutations in the gene are associated with N-Acetylglutamate synthase deficiency.
| v · Metabolism: amino acid metabolism · urea cycle enzymes |
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| Main cycle |
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| Regulatory/transport |
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mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m
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k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon
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m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)
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| v · d · eMitochondrial proteins |
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| Outer membrane |
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| Intermembrane space |
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| Inner membrane |
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| Matrix |
citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase)
anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex)
urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase)
alcohol metabolism (ALDH2)
PMPCB
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| Other/to be sorted |
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| Mitochondrial DNA |
Complex I (MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6) - Complex III (MT-CYB) - Complex IV (MT-CO1, MT-CO2, MT-CO3)
ATP synthase (MT-ATP6, MT-ATP8)
tRNA ( MT-TA, MT-TC, MT-TD, MT-TE, MT-TF, MT-TG, MT-TH, MT-TI, MT-TK, MT-TL1, MT-TL2, MT-TM, MT-TN, MT-TP, MT-TQ, MT-TR, MT-TS1, MT-TS2, MT-TT, MT-TV, MT-TW, MT-TY)
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see also mitochondrial diseases
B strc: edmb (perx), skel (ctrs), epit, cili, mito, nucl (chro)
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