3-sulfino-L-alanine

Three steps

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACAA1 and 1 moremitochondrial matrix, cytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-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 (gamma-linolenate)

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

• butanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by SLC25A12, SLC25A13, NAT8L, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

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

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

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

• eicosanoyl-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 (eicosanoate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SUCLG1 and 2 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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)-octadecadienoic acid)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by SLC25A12, SLC25A13, NAT8L, CROTmitochondrial matrix, cytosolOne of 20 routes

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

  Catalysed by SLC25A12, SLC25A13, NAT8Lmitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-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 (stearate)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 6 moremitochondrial matrix, cytosolOne of 15 routes

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

  Catalysed by SLC25A12, SLC25A13, AGXT2, SLC7A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. 3-sulfino-L-alanine carboxy-lyase
  2. Hypotaurine Oxidase, Extracellular
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by GAD1, CSAD, MTO1cytosol, extracellular space

• carnosineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC25A12, SLC25A13, GAD2, CARNS1mitochondrial matrix, cytosol

• L-dopaReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, SLC7A8mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• 3-guanidinopropanoateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by SLC25A12, SLC25A13, GAD2, GATMmitochondrial matrix, cytosol

• L-histidineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, SLC7A8mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by GOT1, MPSTcytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by GOT1, AGXT2cytosol

• sphing-4-enineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by SLC25A12, SLC25A13, NAT8L, ASAH1 and 3 moremitochondrial matrix, cytosol, Golgi apparatusOne of 2 routes

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

  Catalysed by SLC25A12, SLC25A13, AGXT2, SLC7A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SAT1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

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

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

• dehydroepiandrosterone 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. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

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

• L-dopaquinoneReach 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. L-tyrosine + O2 = L-dopaquinone + H2O

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

• glutathioneReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GOT1, TSTD1cytosolOne of 2 routes

• L-threonineReach 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. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, SLC1A5mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-kynurenineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. Transport of L-Kynurenine

  Catalysed by SLC25A12, SLC25A13, SLC7A13, SLC43A1mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 5 routes

• dimethylamineReach 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. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by SLC25A12, SLC25A13, AGXT2, DDAH1mitochondrial matrix, cytosolOne of 2 routes

• IMPReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. IMP:L-aspartate ligase (GDP-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by SLC25A12, SLC25A13, ADSS2, ADSS1mitochondrial matrix, cytosol

• taurocyamineReach from here →
  1. 3-sulfino-L-alanine carboxy-lyase
  2. Hypotaurine Oxidase, Extracellular
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by GAD1, CSAD, GATMcytosol, extracellular space

• L-cysteateReach from here →
  1. 3-sulfino-L-alanine carboxy-lyase
  2. Hypotaurine Oxidase, Extracellular
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by GAD1, CSAD, GADL1cytosol, extracellular space

• 3-hydroxy-L-kynurenineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

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

• L,L-cystathionineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by SLC25A12, SLC25A13, NAT8L, NAGSmitochondrial matrix, cytosol

• L-homocysteineReach 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. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, BHMT2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• carbamoyl phosphateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

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

• adenosine 5'-phosphosulfateReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC25A12, SLC25A13, SLC25A14, SLC25A30 and 2 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• S-methyl-L-methionineReach 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. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, BHMT2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• glutathione disulfideReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  3. cystine to cysteine conversion

  Catalysed by SLC25A12, SLC25A13, SLC7A13mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• an acyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by SLC25A12, SLC25A13, NAT8L, ASAH1 and 3 moremitochondrial matrix, cytosol, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  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 SLC25A12, SLC25A13, AGXT2, SLC1A4mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-2-aminopentanoateReach 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. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by SLC25A12, SLC25A13, AGXT2mitochondrial matrix, cytosol

• L-2-aminohexanoateReach 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. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by SLC25A12, SLC25A13, AGXT2mitochondrial matrix, cytosol

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by SLC25A12, SLC25A13, PAICS, ADSLmitochondrial matrix, cytosolOne of 2 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

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

• N-formyl-L-kynurenineReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by SLC25A12, SLC25A13, SLC7A13, IDO1 and 2 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• (3S)-citramalyl-CoAReach from here →
  1. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  2. 3-sulfinylpyruvic acid hydrolysis
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by GOT1, CLYBLcytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by SLC25A12, SLC25A13, NAT8L, SLC27A2 and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach 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. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by SLC25A12, SLC25A13, SLC7A13, PPATmitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes