malonic acid

Three steps

• 7,8-dihydrofolateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. Folate Transport by Oats
  3. dihydrofolate:NAD+ oxidoreductase

  Catalysed by SLC25A10, SLC22A8, SLC22A7, DHFR and 1 moremitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and glycine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by SLC6A9, SLC6A14, SHMT1, SHMT2extracellular space, cytosol

• (13S)-hydroperoxy-(9Z,11E)-octadecadienoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. sulfite:ferricytochrome-c oxidoreductase
  3. 13(S)-HPODE to 13-oxy-radical-octadecadienoate conversion

  Catalysed by SLC25A10, SUOXmitochondrial matrix, cytosol

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by SLC25A10, ASLmitochondrial matrix, cytosolOne of 6 routes

• (R)-S-lactoylglutathioneReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (R)-S-lactoylglutathione = methylglyoxal + glutathione

  Catalysed by SLC25A10, TSTD1, GLO1mitochondrial matrix, cytosol

• carnosineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC6A6, SLC6A14, CARNS1extracellular space, cytosol

• 4-guanidinobutanoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of arginine and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by SLC6A14, GATMextracellular space, cytosolOne of 2 routes

• L-dopaReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)

  Catalysed by SLC25A10, ADSL, TNRC6Bmitochondrial matrix, cytosolOne of 4 routes

• O-acetyl-(R)-carnitineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Carnitine Transport by Atb0
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by SLC6A14, CPT1A, CPT1C, CPT1Bextracellular space, cytosolOne of 9 routes

• 3-guanidinopropanoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of arginine and chloride (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by SLC6A14, GATMextracellular space, cytosolOne of 2 routes

• L-histidineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• an N-acylglycineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by SLC6A9, SLC6A14, GLYATL3, GLYAT and 1 moreextracellular space, cytosol

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by SLC25A10, MPST, TSTmitochondrial matrix, cytosolOne of 4 routes

• S-formylglutathioneReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. S-formylglutathione + H2O = formate + glutathione + H(+)

  Catalysed by SLC25A10, TSTD1, ESDmitochondrial matrix, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by SLC25A10, MPST, TST, AGXT2mitochondrial matrix, cytosol

• succinate semialdehydeReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A12, ABATextracellular space, cytosol

• 3',5'-cyclic GMPReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-threonineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of alanine and chloride (cytosol to extracellular)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by SLC6A14, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• adenosine 5'-phosphoramidateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. ATP:sulfate adenylyltransferase
  3. adenosine 5'-phosphosulfate + NH4(+) = adenosine 5'-phosphoramidate + sulfate + 2 H(+)

  Catalysed by SLC25A10, PAPSS1, PAPSS2, FHITmitochondrial matrix, cytosol

• L-dopaquinoneReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by SLC6A14, TYRextracellular space, cytosol

• L-threonineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Glutamine by Atb0 Transporter
  3. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)

  Catalysed by SLC6A14, SLC1A5extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by SLC6A14, AASSextracellular space, cytosol, mitochondrial matrix

• L-kynurenineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Transport of L-Kynurenine

  Catalysed by SLC6A14, SLC7A8extracellular space, cytosolOne of 5 routes

• leukotriene C4Reach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. leukotriene C4 = leukotriene A4 + glutathione

  Catalysed by SLC25A10, TSTD1, MGST3, GSTM4 and 2 moremitochondrial matrix, cytosolOne of 2 routes

• taurocyamineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by SLC6A6, GATMextracellular space, cytosolOne of 2 routes

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by SLC25A10, SLC25A14, SLC25A30, AGAmitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• L-cysteateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SLC6A6, GADL1, CSADextracellular space, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of alanine and chloride (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SLC6A14, KYNUextracellular space, cytosol

• 3',5'-cyclic AMPReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic AMP(out) + a dicarboxylate(in) = 3',5'-cyclic AMP(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• gamma-L-glutamyl-L-cysteineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by SLC6A14, GCLC, GCLMextracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by SLC6A14extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC6A14, BHMT2extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Glutamine by Atb0 Transporter
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by SLC6A14, CADextracellular space, cytosolOne of 2 routes

• N,N-dimethylglycineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of betaine and chloride (cytosol to extracellular)
  3. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by SLC6A12, BHMTextracellular space, cytosol

• S-methyl-L-methionineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC6A14, BHMT2extracellular space, cytosol

• glutathione disulfideReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by SLC25A10, SLC25A14, SLC25A30, SLC26A1 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 23 routes

• L-tyrosineReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)

  Catalysed by SLC25A10, SLC25A14, SLC25A30, SLC7A13mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by SLC25A10, SLC25A14, SLC25A30, AGXT2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of alanine and chloride (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by SLC6A14, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-oxo-L-prolineReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by SLC25A10, TSTD1, CHAC2, CHAC1mitochondrial matrix, cytosol

• histamineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and histidine (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by SLC6A14, HDCextracellular space, cytosol

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by SLC25A10, ADSLmitochondrial matrix, cytosolOne of 4 routes

• L-thyroxineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and iodide (cytosol to extracellular)
  3. 3,3',5-triiodo-L-thyronine,iodide:acceptor oxidoreductase (iodinating)

  Catalysed by SLC26A4, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• kynurenateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A8mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by SLC6A14, IL4I1extracellular space, cytosol

• S-sulfanyl-L-cysteineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by SLC6A14, CARS2extracellular space, cytosol

• N-formyl-L-kynurenineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Tryptophan by Atb0 Transporter
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by SLC6A14, IDO1, TDO2, IDO2extracellular space, cytosol

• (3S)-citramalyl-CoAReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by SLC25A10, MPST, TST, CLYBLmitochondrial matrix, cytosol

• 5-phospho-beta-D-ribosylamineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Glutamine by Atb0 Transporter
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by SLC6A14, PPATextracellular space, cytosolOne of 2 routes