isocitric acid

Three steps

• trans-4-hydroxy-L-prolineReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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 2 routes

• kynurenateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-kynurenine + glyoxylate = kynurenate + glycine + H2O

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

• dCDPReach from here →
  1. transport of isocitrate 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. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

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

• 5-amino-2-oxopentanoateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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

• S-sulfanylglutathioneReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by SLC25A1, SLC25A10, STSmitochondrial matrix, cytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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 isocitrate and PEP (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. dTTP + H2O = dTMP + diphosphate + H(+)

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

• ubiquinol-10Reach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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

• xanthurenateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  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

• 3-methylmalateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by SLC25A1, CLYBLmitochondrial matrix, cytosol

• 3-deoxy-D-glycero-beta-D-galacto-non-2-ulopyranosonate 9-phosphateReach from here →
  1. citrate hydroxymutase
  2. phosphoenolpyruvate(in) + citrate(out) = phosphoenolpyruvate(out) + citrate(in)
  3. aldehydo-D-mannose 6-phosphate + phosphoenolpyruvate + H2O = 3-deoxy-D-glycero-beta-D-galacto-non-2-ulopyranosonate 9-phosphate + phosphate

  Catalysed by ACO1, SLC25A1, NANScytosol, inner side of the membrane, outer side of the membrane

• thiamine(1+) diphosphateReach from here →
  1. transport of isocitrate and PEP (cytosol to mitochondria)
  2. dATP:pyruvate 2-O-phosphotransferase
  3. Antiport of thiamine-diphosphate and dATP

  Catalysed by SLC25A1, PKM, PKLR, SLC25A19mitochondrial matrix, cytosolOne of 2 routes

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

  Catalysed by SLC25A1, ME2, ME3, PDHA1 and 2 moremitochondrial matrix, cytosol