malonic acid

Three steps

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

  Catalysed by SLC25A10, SGSHmitochondrial matrix, cytosol

• D-glucosamine 6-phosphateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Glutamine by Atb0 Transporter
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by SLC6A14, GFPT2, GFPT1extracellular space, cytosol

• L-methionine (S)-S-oxideReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC6A14, MSRAextracellular space, cytosol

• L-methionine (R)-S-oxideReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC6A14, MSRB3, MSRB1, MSRB2extracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

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

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by SLC6A9, SLC6A14, GLYATL2extracellular space, cytosolOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by SLC6A14, PPCSextracellular space, cytosolOne of 4 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-iodo-L-tyrosineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. thyroid peroxidase (iodine)

  Catalysed by SLC6A14, TPOextracellular space, cytosolOne of 2 routes

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

• prostaglandin E2Reach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. prostaglandin E2(out) + a dicarboxylate(in) = prostaglandin E2(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

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

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

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

  Catalysed by SLC25A10, SLC25A14, SLC25A30, SLC25A12 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• L-cysteinylglycineReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glutathione + H2O = L-cysteinylglycine + L-glutamate

  Catalysed by SLC25A10, TSTD1, GGT3P, GGT1 and 3 moremitochondrial matrix, cytosolOne of 4 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by SLC25A10, MPST, TST, HOGA1mitochondrial matrix, cytosol

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by CLTRN, SLC6A20, PYCR1, PYCR3 and 1 moreextracellular space, cytosolOne of 4 routes

• 3-demethylubiquinol-10Reach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate
  3. 3-demethylubiquinol-10 + S-adenosyl-L-methionine = ubiquinol-10 + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SLC25A10, SQOR, COQ3mitochondrial matrix, cytosol

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of arginine and chloride (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by SLC6A14, NOS3, NOS1, NOS2extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of alanine and chloride (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC6A14, MOCOSextracellular space, cytosol

• (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A14extracellular space, cytosol, endoplasmic reticulum

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by SLC25A10, MPST, TST, HOGA1mitochondrial matrix, cytosol

• (2S)-2-aminobutanoateReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and glycine (cytosol to extracellular)
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by SLC6A9, SLC6A14, AGXT2extracellular space, cytosolOne of 2 routes

• O-dodecanoyl-R-carnitineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Carnitine Transport by Atb0
  3. carnitine O-palmitoyltransferase (dodecanoylcarnitine)

  Catalysed by SLC6A14, CPT1A, CPT1C, CPT1Bextracellular space, cytosolOne of 7 routes

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (9S)-hydroperoxy-(10E,12Z)-octadecadienoate + 2 glutathione = (9S)-hydroxy-(10E,12Z)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX4mitochondrial matrix, cytosol

• thio-Mo-molybdopterinReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC6A14, MOCOSextracellular space, cytosol

• glycodeoxycholateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glycodeoxycholate(in) + glutathione(in) + ATP + H2O = glycodeoxycholate(out) + glutathione(out) + ADP + phosphate + H(+)

  Catalysed by SLC25A10, TSTD1, ABCC4mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX2 and 1 moremitochondrial matrix, cytosol

• O-4,8-dimethylnonanoyl-(R)-carnitineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Carnitine Transport by Atb0
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by SLC6A14, CRAT, CROTextracellular space, cytosol, peroxisomeOne of 5 routes

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

  Catalysed by SLC6A9, SLC6A14, GLYATL2extracellular space, cytosol

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

  Catalysed by SLC6A9, SLC6A14, BAATextracellular space, cytosol

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

  Catalysed by SLC6A9, SLC6A14, BAATextracellular space, cytosol

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

  Catalysed by SLC6A9, SLC6A14, BAATextracellular space, cytosol

• 16alpha-hydroxydehydroepiandrosterone 3-sulfateReach from here →
  1. transport of malonate and sulfate (cytosol to mitochondria)
  2. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
  3. dehydroepiandrosterone 3-sulfate + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha-hydroxydehydroepiandrosterone 3-sulfate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by SLC25A10, STS, CYP3A7mitochondrial matrix, cytosol

• an N(2)-acyl-L-glutamineReach from here →
  1. Malonate Transport, Antiport with Chloride
  2. Transport of L-Glutamine by Atb0 Transporter
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by SLC6A14, GLYATL1B, GLYATL1extracellular space, cytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, PTGES, MGST3 and 4 moremitochondrial matrix, cytosol

• (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX4mitochondrial matrix, cytosol

• an S-substituted glutathioneReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by SLC25A10, TSTD1, GSTT4, GSTA4 and 21 moremitochondrial matrix, cytosolOne of 2 routes

• (13S)-hydroxy-(9Z,11E)-octadecadienoateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (13S)-hydroperoxy-(9Z,11E)-octadecadienoate + 2 glutathione = (13S)-hydroxy-(9Z,11E)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GSTA1 and 2 moremitochondrial matrix, cytosol

• taurochenodeoxycholateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. taurochenodeoxycholate(in) + glutathione(in) + ATP + H2O = taurochenodeoxycholate(out) + glutathione(out) + ADP + phosphate + H(+)

  Catalysed by SLC25A10, TSTD1, ABCC4mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. transport of malonate and sulfite (cytosol to mitochondria)
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

  Catalysed by SLC25A10, MPST, TST, PDHA1 and 2 moremitochondrial matrix, cytosol