citric acid

Three steps

• dehydroepiandrosterone 3-sulfateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by SLC25A1, SLC25A10, STSmitochondrial matrix, cytosol

• L-tryptophanReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Transport of L-Tryptophan by Atb0 Transporter

  Catalysed by SLC25A1, SLC26A6, SLC6A14mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• glutathioneReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfite (cytosol to mitochondria)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by SLC25A1, SLC25A10, TSTD1mitochondrial matrix, cytosolOne of 2 routes

• beta-alanineReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. transport of beta-alanine and chloride (cytosol to extracellular)

  Catalysed by SLC25A1, SLC26A6, SLC6A6, SLC6A14mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• L-alanineReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by SLC25A1, ME2, ME3, AGXT2mitochondrial matrix, cytosolOne of 22 routes

• L-asparagineReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Transport of L-Asparagine by Atb0 Transporter

  Catalysed by SLC25A1, SLC26A6, SLC6A14mitochondrial matrix, cytosol, extracellular spaceOne of 5 routes

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. ATP citrate lyase (citrate)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by ACLY, GOT2, AGAcytosol, mitochondrial matrix

• L-phenylalanineReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Transport of L-Phenylalanine by Atb0 Transporter

  Catalysed by SLC25A1, SLC26A6, SLC6A14mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• L,L-cystathionineReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ACLY, CS, NAGScytosol, mitochondrial matrix

• adenosine 5'-phosphosulfateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC25A1, SLC25A10, PAPSS1, PAPSS2mitochondrial matrix, cytosolOne of 4 routes

• (2R)-3-phosphoglycerateReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)
  3. D-phosphoglycerate 2,3-phosphomutase

  Catalysed by SLC25A1, ENO1, ENO2, ENO3 and 4 moremitochondrial matrix, cytosolOne of 2 routes

• glutathione disulfideReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by SLC25A1, SLC25A10, TSTD1mitochondrial matrix, cytosol

• L-tyrosineReach from here →
  1. ATP citrate lyase (citrate)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)

  Catalysed by ACLY, GOT2, SLC7A13cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. ATP citrate lyase (citrate)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by ACLY, GOT2, AGXT2cytosol, mitochondrial matrixOne of 7 routes

• an acyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ACLY, CS, ASAH1, ACER2 and 2 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by SLC25A1, SDHB, SDHA, PRODH2mitochondrial matrix, cytosol

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by SLC25A1, FH, ADSLmitochondrial matrix, cytosolOne of 4 routes

• kynurenateReach from here →
  1. Citrate Transport, Mitochondrial
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-kynurenine + glyoxylate = kynurenate + glycine + H2O

  Catalysed by SLC25A1, CLYBL, KYAT3, AADATmitochondrial matrix, cytosol

• (3Z)-dodecenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by ACLY, CS, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• dCDPReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. dATP:pyruvate 2-O-phosphotransferase
  3. cytidine/uridine monophosphate kinase (dADP)

  Catalysed by SLC25A1, PKM, PKLR, CMPK1mitochondrial matrix, cytosolOne of 4 routes

• (3S)-citramalyl-CoAReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by SLC25A1, ME2, ME3, CLYBLmitochondrial matrix, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ACLY, CS, SLC27A2, SLC27A5cytosol, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-amino-2-oxopentanoateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by SLC25A1, ME2, ME3, AGXT2mitochondrial matrix, cytosolOne of 2 routes

• deoxycholoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ACLY, CS, SLC27A2, SLC27A5cytosol, mitochondrial matrixOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfite (cytosol to mitochondria)
  3. S-sulfanylglutathione + O2 + H2O = sulfite + glutathione + 2 H(+)

  Catalysed by SLC25A1, SLC25A10, ETHE1mitochondrial matrix, cytosolOne of 2 routes

• 4-aminobutanoateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by SLC25A1, SLC26A6, SLC6A12mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• L-prolineReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)

  Catalysed by SLC25A1, SDHB, SDHA, PRODH and 1 moremitochondrial matrix, cytosol

• estrone 3-sulfateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by SLC25A1, SLC25A10, STSmitochondrial matrix, cytosol

• 3-sulfino-L-alanineReach from here →
  1. ATP citrate lyase (citrate)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by ACLY, GOT2, SLC25A12, SLC25A13cytosol, mitochondrial matrixOne of 4 routes

• decanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ACLY, CS, ACSM4, ACSM1 and 4 morecytosol, mitochondrial matrixOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ACLY, CS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 11 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by SLC25A1, ME2, ME3, HOGA1mitochondrial matrix, cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ACLY, CS, CPT1A, CPT1Bcytosol, mitochondrial matrix

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by SLC25A1, SLC25A10, SLC4A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• dTMPReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. dTTP + H2O = dTMP + diphosphate + H(+)

  Catalysed by SLC25A1, PKM, PKLR, ASMTLmitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• ubiquinol-10Reach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfite (cytosol to mitochondria)
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by SLC25A1, SLC25A10, SQORmitochondrial matrix, cytosol

• hexacosanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 10 routes

• docosanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• xanthurenateReach from here →
  1. Citrate Transport, Mitochondrial
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by SLC25A1, CLYBL, KYAT3, AADATmitochondrial matrix, cytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ACLY, CS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by SLC25A1, ME2, ME3, HOGA1mitochondrial matrix, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes