N-carbamoyl-L-aspartic acid

Three steps

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate

  Catalysed by CAD, AGXT2cytosolOne of 2 routes

• nonadecanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Activation of dodecanedioic acid

  Catalysed by CAD, NAT8Lcytosol, mitochondrial matrixOne of 4 routes

• decanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Sebacoylcarnitine

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

• octanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Suberylcarnitine

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by CAD, NAT8Lcytosol, mitochondrial matrixOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrix

• (13Z)-eicosenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• N-acetyl-L-aspartyl-L-glutamateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + L-glutamate + ATP = N-acetyl-L-aspartyl-L-glutamate + ADP + phosphate + H(+)

  Catalysed by CAD, NAT8L, RIMKLA, RIMKLBcytosol, mitochondrial matrix

• N-acetyl-L-aspartyl-L-glutamyl-L-glutamateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + 2 L-glutamate + 2 ATP = N-acetyl-L-aspartyl-L-glutamyl-L-glutamate + 2 ADP + 2 phosphate + 2 H(+)

  Catalysed by CAD, NAT8L, RIMKLAcytosol, mitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by CAD, NAT8L, CRAT, CROTcytosol, mitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by CAD, NAT8L, ACAA1, HSD17B4 and 2 morecytosol, mitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Hexadecanedioylcarnitine

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 2 routes

• pristanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. fatty-acid-CoA ligase

  Catalysed by CAD, NAT8L, SLC27A2cytosol, mitochondrial matrixOne of 2 routes

• undecanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by CAD, NAT8L, ACSM4, ACSM1 and 4 morecytosol, mitochondrial matrixOne of 3 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by CAD, NAT8L, ACADM, ECHS1 and 2 morecytosol, mitochondrial matrix

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by CAD, NAT8L, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrix

• an N(2)-acyl-L-glutamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by CAD, SLC7A13, GLYATL1B, GLYATL1cytosol, inner side of the membrane, outer side of the membrane

• (7Z)-hexadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 4 routes