citric acid

Three steps

• malonic acidReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. transport of malonate and sulfate (cytosol to mitochondria)

  Catalysed by SLC25A1, SLC25A10mitochondrial matrix, cytosolOne of 6 routes

• 3-hydroxypyruvic acidReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. L-Serine:pyruvate aminotransferase

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

• (S)-dihydroorotateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

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

• glutarateReach from here →
  1. citrate hydro-lyase (cis-aconitate-forming)
  2. cis-aconitate carboxy-lyase
  3. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate

  Catalysed by ACO2, ACOD1, SUGCTmitochondrial matrix

• (2R,3R)-tartrateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by SLC25A1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by SLC25A1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by SLC25A1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by SLC25A1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-carbamoyl-L-aspartateReach from here →
  1. ATP citrate lyase (citrate)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by ACLY, GOT2, CADcytosol, mitochondrial matrix

• 3-oxopropanoateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by SLC25A1, ME2, ME3, AGXT2mitochondrial matrix, cytosol

• 2-oxohexanoateReach from here →
  1. ATP citrate lyase (citrate)
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by ACLY, AGXT2cytosol

• D-serineReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. D-serine = pyruvate + NH4(+)

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

• N-acetylneuraminateReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. N-acetylneuraminate synthase
  3. N-acetylneuraminate 9-phosphate + H2O = N-acetylneuraminate + phosphate

  Catalysed by SLC25A1, NANS, NANPmitochondrial matrix, cytosol

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. Citrate Transport, Mitochondrial
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by SLC25A1, CLYBL, AADATmitochondrial matrix, cytosol

• D-glucopyranose 6-phosphateReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. dATP:pyruvate 2-O-phosphotransferase
  3. dATP:D-glucose 6-phosphotransferase

  Catalysed by SLC25A1, PKM, PKLR, HK1 and 3 moremitochondrial matrix, cytosol

• (R)-lactic acidReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-Lactate:NAD+ oxidoreductase

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

• (S)-lactic acidReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC25A1, ME2, ME3, LDHB and 1 moremitochondrial matrix, cytosolOne of 5 routes

• 2-iminopropanoateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

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

• N-acetyl-beta-neuraminic acidReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

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

• taurineReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC25A1, SLC26A6, SLC6A6mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamideReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol

  Catalysed by SLC25A1, SDHB, SDHA, NQO2mitochondrial matrix, cytosol

• a 3-acylpyruvateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by SLC25A1, ME2, ME3, FAHD1mitochondrial matrix, cytosol

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

  Catalysed by SLC25A1, CLYBL, AADATmitochondrial matrix, cytosolOne of 28 routes

• (R)-methylmalonyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by ACLY, CS, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by ACLY, CScytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ACLY, CS, ACP2cytosol, mitochondrial matrix, lysosomeOne of 2 routes

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

  Catalysed by ACLY, CScytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ACLY, CS, ACAA1, MYO5Bcytosol, mitochondrial matrix, peroxisomeOne of 2 routes

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

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

• butanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by ACLY, CS, ACSM4, ACSM1 and 4 morecytosol, mitochondrial matrixOne of 9 routes

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

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

• glutaryl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Production of Glutaryl Carnitine

  Catalysed by ACLY, CS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 12 routes

• itaconyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ACLY, CS, SUCLG1, SUCLG2 and 1 morecytosol, mitochondrial matrixOne of 6 routes

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

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 13 routes

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

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

• octanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ACLY, CS, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. phytanate-CoA ligase

  Catalysed by ACLY, CScytosol, mitochondrial matrixOne of 5 routes

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

  Catalysed by ACLY, CS, SLC27A2, ACSL4 and 5 morecytosol, mitochondrial matrixOne of 15 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

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

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

  Catalysed by SLC25A1, CLYBL, AADATmitochondrial matrix, cytosol

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)

  Catalysed by SLC25A1, FH, ADSL, TNRC6Bmitochondrial matrix, cytosolOne of 4 routes

• dGMPReach from here →
  1. transport of citrate and PEP (cytosol to mitochondria)
  2. dGTP:pyruvate 2-O-phosphotransferase
  3. 2-Deoxyguanosine 5-Triphosphate Pyrophosphohydrolase

  Catalysed by SLC25A1, PKM, PKLR, ITPAmitochondrial matrix, cytosol, extracellular space

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

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

• 2-methyl-3-oxopropanoateReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by SLC25A1, ME2, ME3, AGXT2mitochondrial matrix, cytosol

• guanidinoacetateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Transport of Guanidinoacetic Acid

  Catalysed by SLC25A1, SLC26A6mitochondrial matrix, cytosol, extracellular space

• sphing-4-enineReach 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

• spermidineReach from here →
  1. ATP citrate lyase (citrate)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by ACLY, CS, SAT1, SAT2cytosol, mitochondrial matrixOne of 3 routes

• L-methionineReach from here →
  1. Citrate Transport, Mitochondrial
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by SLC25A1, ME2, ME3, TYW1B and 1 moremitochondrial matrix, cytosolOne of 6 routes

• N-sulfo-D-glucosamineReach from here →
  1. Citrate Transport, Mitochondrial
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC25A1, SLC25A10, SGSHmitochondrial matrix, cytosol