L-methionine

Three steps

• taurodeoxycholateReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by NAGS, SLC27A2, SLC27A5, BAATmitochondrial matrix, cytosolOne of 4 routes

• 3-hydroxyanthranilateReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SLC7A5, KYNUextracellular space, cytosol

• N-formylanthranilic acidReach from here →
  1. transport of methionine and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

  Catalysed by SLC7A5, IDO1, TDO2, IDO2 and 1 moreextracellular space, cytosol

• cholesteryl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, endoplasmic reticulum

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• CHEBI:36882Reach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2 and 1 moremitochondrial matrix

• 3-hydroxyisovaleric acidReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by NAGS, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• sphingosine 1-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• 7alpha,25-dihydroxycholesterolReach from here →
  1. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. cytochrome P450 (25-hydroxycholesterol)

  Catalysed by CDKAL1, CH25H, CYP7B1endoplasmic reticulumOne of 2 routes

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• D-glucopyranose 6-phosphateReach from here →
  1. transport of glutamine and methionine (cytosol to extracellular)
  2. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by SLC7A5, CAD, G6PC2extracellular space, cytosol, endoplasmic reticulum

• (R)-lactic acidReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC7A5, AGXT2, LDHDextracellular space, cytosol, mitochondrial matrix

• (S)-lactic acidReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC7A5, AGXT2, LDHB, LDHAL6Bextracellular space, cytosol, mitochondrial matrixOne of 7 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Production of Glutaryl Carnitine
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• (6R)-L-erythro-6,7-dihydrobiopterinReach from here →
  1. transport of methionine and tryptophan (cytosol to extracellular)
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L-erythro-6,7-dihydrobiopterin + H2O

  Catalysed by SLC7A5, TPH1, TPH2, PCBD1 and 1 moreextracellular space, cytosol

• 2-iminopropanoateReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by SLC7A5, AGXT2, RIDAextracellular space, cytosol, mitochondrial matrix

• phosphoenolpyruvic acidReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. ATP citrate lyase (citrate)
  3. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by NAGS, ACLY, PCK1mitochondrial matrix, cytosolOne of 3 routes

• 13-cis-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 3 moremitochondrial matrix, cytosol

• spermineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

  Catalysed by NAGS, SAT1, SAT2, SMSmitochondrial matrix, cytosolOne of 2 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by SLC7A5, AGXT2, NPLextracellular space, cytosol, mitochondrial matrix

• tert-butanolReach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by SLC7A5, GSS, CLIC2, GPX1 and 3 moreextracellular space, cytosol

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 2 moremitochondrial matrix, cytosol, endoplasmic reticulum

• N-(acetyl)-sphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• a 1,3-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 3 moremitochondrial matrix, Golgi apparatus

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of methionine and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by SLC7A5, BCAT2, BCKDHA, BCKDHBextracellular space, cytosol, mitochondrial matrix

• 3-(methylsulfanyl)propanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 3-(methylsulfanyl)propanoate + CO + formate + 2 H(+)

  Catalysed by AADAT, ADI1

• a 1,2-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• an N-acylsphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 7 moremitochondrial matrix, Golgi apparatus

• N-tetracosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

• N-hexacosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

• malonaldehydeReach from here →
  1. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. thromboxane A synthase (12(S)-HHT)

  Catalysed by CDKAL1, PTGS1, PTGS2, TBXAS1cytosolOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus

• a 3-acylpyruvateReach from here →
  1. transport of alanine and methionine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by SLC7A5, AGXT2, FAHD1extracellular space, cytosol, mitochondrial matrix

• succinyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-methylmalonyl-CoA = succinyl-CoA

  Catalysed by NAGS, ALDH6A1, MMUTmitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by NAGS, GLYAT, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

• (2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• acryloyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Propanoate:CoA ligase (AMP-forming)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by NAGS, ACSS3, ACSS2, ACSS1 and 2 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• benzoyl-CoAReach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC7A5, GLYATextracellular space, cytosol, mitochondrial matrix

• hexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• propanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetylmethionine)
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by NAGS, ECHDC1mitochondrial matrix

• prostaglandin E1Reach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 4 routes

• prostaglandin I2Reach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 4 routes

• prostaglandin F2alphaReach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 4 routes

• prostaglandin D2Reach from here →
  1. transport of glycine and methionine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by SLC7A5, GSS, SLCO2A1extracellular space, cytosolOne of 5 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  2. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O
  3. (15S)-15-Hydroxy-5,8,11-cis-13-trans-icosatetraenoate:NADP+ 15-oxidoreductase

  Catalysed by CDKAL1, TBXAS1, PTGS2, PTGIScytosolOne of 3 routes