5-(3,3-dimethylguanidino)-2-oxopentanoate

Three steps

• (2S)-2-phospholactateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (2S)-2-phospholactate + H2O = (S)-lactate + phosphate

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moreOne of 2 routes

• L-prolineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by AGXT2, SLC1A4mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E2(out) + 2 (S)-lactate(in) = prostaglandin E2(in) + 2 (S)-lactate(out)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by AGXT2mitochondrial matrixOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by AGXT2, SLC25A12, SLC25A13mitochondrial matrix, cytosolOne of 4 routes

• decanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by AGXT2, CS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• L-cysteinylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. gamma-glutamyltransferase (alanine)

  Catalysed by AGXT2, GGTLC3, GGT1, GGT5 and 3 moremitochondrial matrix, cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by AGXT2, CS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• dTMPReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. pyruvate kinase (dTDP)
  3. DTDP Diphosphohydrolase

  Catalysed by AGXT2, PKM, PKLR, ENTPD3 and 2 morecytosol, extracellular spaceOne of 5 routes

• CHEBI:63544Reach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by AGXT2, SLC25A2, NOS3, NOS1 and 1 moreinner side of the membrane, outer side of the membrane, cytosol

• Mo-molybdopterinReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AGXT2, MOCOSmitochondrial matrix

• (2E)-octadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• L-threonylcarbamoyladenylateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. L-threonine = 2-oxobutanoate + NH4(+)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by AGXT2, SDS, SDSL, YRDC

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Activation of dodecanedioic acid

  Catalysed by AGXT2, CSmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Production of Sebacoylcarnitine

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Production of Suberylcarnitine

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by AGXT2, CSmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by AGXT2, CS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. D-serine = pyruvate + NH4(+)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by AGXT2, SRR

• beta-citrylglutamateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. citrate + L-glutamate + ATP = beta-citrylglutamate + ADP + phosphate + H(+)

  Catalysed by AGXT2, CS, RIMKLBmitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by AGXT2, CS, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by AGXT2, CS, ACAA1, HSD17B4 and 2 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by AGXT2, CS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes