3-sulfino-L-alanine

Three steps

• phosphoenolpyruvateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by SLC25A12, SLC25A13, GOT2, PCK1 and 1 moremitochondrial matrix, cytosol

• D-glucosamine 6-phosphateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by SLC25A12, SLC25A13, SLC7A13, GFPT2 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• L-methionine (S)-S-oxideReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC25A12, SLC25A13, SLC7A13, MSRAmitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• L-methionine (R)-S-oxideReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by SLC25A12, SLC25A13, SLC7A13, MSRB3 and 2 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• 5-amino-2-oxopentanoateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by GOT1, AGXT2cytosol

• deoxycholoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 1 moremitochondrial matrix, cytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. S-sulfanylglutathione + O2 + H2O = sulfite + glutathione + 2 H(+)

  Catalysed by GOT1, ETHE1cytosol

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by SLC25A12, SLC25A13, SLC7A13, PAHmitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• L-prolineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by SLC25A12, SLC25A13, AGXT2, SLC1A4mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• estrone 3-sulfateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by SLC25A12, SLC25A13, SLC25A14, SLC25A30 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• decanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 11 routes

• L-cysteinylglycineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. gamma-glutamyltransferase (alanine)

  Catalysed by SLC25A12, SLC25A13, AGXT2, GGTLC3 and 5 moremitochondrial matrix, cytosolOne of 2 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by GOT1, HOGA1cytosol

• 3-hydroxyisovaleryl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 1 moremitochondrial matrix, cytosol

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by SLC25A12, SLC25A13, SLC25A14, SLC25A30 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 2 routes

• CHEBI:63544Reach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• ubiquinol-10Reach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by GOT1, SQORcytosol

• hexacosanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• Mo-molybdopterinReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC25A12, SLC25A13, AGXT2, MOCOSmitochondrial matrix, cytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by GOT1, HOGA1cytosol

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate

  Catalysed by SLC25A12, SLC25A13, AGXT2mitochondrial matrix, cytosolOne of 2 routes

• nonadecanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Activation of dodecanedioic acid

  Catalysed by SLC25A12, SLC25A13, NAT8Lmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Sebacoylcarnitine

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Suberylcarnitine

  Catalysed by SLC25A12, SLC25A13, NAT8L, CPT1A and 2 moremitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by SLC25A12, SLC25A13, NAT8Lmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes