carbamoyl phosphate

Three steps

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by OTC, SLC7A6, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• nonadecanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• NAADP(+)Reach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-glucose-6-phosphate:NADP+ 1-oxidoreductase
  3. NAADP(+) + D-glucose 6-phosphate = NAADPH + 6-phospho-D-glucono-1,5-lactone + H(+)

  Catalysed by G6PC2, G6PDendoplasmic reticulum

• dodecanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Activation of dodecanedioic acid

  Catalysed by CADcytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Production of Sebacoylcarnitine

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Production of Suberylcarnitine

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by CADcytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by CAD, CPT1A, CPT1C, CPT1Bcytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. D-serine(in) + L-glutamine(out) + Na(+)(out) = D-serine(out) + L-glutamine(in) + Na(+)(in)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by CAD, SLC1A5, SRRinner side of the membrane, outer side of the membrane

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by CAD, CRAT, CROTcytosol, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by CAD, ACAA1, HSD17B4, EHHADH and 1 morecytosol, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Production of Hexadecanedioylcarnitine

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. fatty-acid-CoA ligase

  Catalysed by CAD, SLC27A2cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by CAD, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 3 routes

• thio-Mo-molybdopterinReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by OTC, SLC7A6, MOCOSmitochondrial matrix, extracellular space, cytosol

• glycodeoxycholateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. Glycodeoxycholate amidohydrolase

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by CAD, ACADM, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrix

• N-tetradecanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by CAD, HADHA, ACAA2, HADHBcytosol, mitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-docosanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-hexacosanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• (7Z)-hexadecenoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 4 routes