L-cysteine

Three steps

• thyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 3'-iodothyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3,3'-diiodothyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3',5'-diiodothyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 3,3',5'-triiodothyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• L-homocysteine thiolactoneReach from here →
  1. transport of cysteine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP = L-homocysteine thiolactone + AMP + diphosphate

  Catalysed by SLC7A5, BHMT2, MARS1extracellular space, cytosolOne of 2 routes

• L-homocysteinyl-AMPReach from here →
  1. transport of cysteine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by SLC7A5, BHMT2, MARS1extracellular space, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

• (2E,4Z,7Z)-decatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial
  3. Fatty Acid Beta Oxidation (C10:2->C10:3), Mitochondrial

  Catalysed by NAGS, ECHS1, ACAA2, HADH and 1 moremitochondrial matrixOne of 3 routes

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (E)-4-hydroxynon-2-enal + glutathione = 3-(glutathion-S-yl)-4-hydroxynonanal

  Catalysed by CTH, TSTD1, GSTP1cytosol

• ursodeoxycholate 3-sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and ursodeoxycholate (cytosol to extracellular)
  3. ursodeoxycholate + 3'-phosphoadenylyl sulfate = ursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT2A1cytosol, extracellular space

• tauroursodeoxycholate 3-sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and tauroursodeoxycholate (cytosol to extracellular)
  3. tauroursodeoxycholate + 3'-phosphoadenylyl sulfate = tauroursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT2A1cytosol, extracellular space

• glycoursodeoxycholate 3-sulfateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. glycine to glycoursodeoxycholate conversion
  3. glycoursodeoxycholate + 3'-phosphoadenylyl sulfate = glycoursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SULT2A1extracellular space, cytosol

• glycochenodeoxycholate 3-sulfateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)
  3. glycochenodeoxycholate + 3'-phosphoadenylyl sulfate = glycochenodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, BAAT and 1 moreextracellular space, cytosol

• deoxycholateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. Glycodeoxycholate amidohydrolase
  3. Glycodeoxycholate amidohydrolase

  Catalysed by SLC3A2, SLC7A5, SLC7A8extracellular space, cytosolOne of 5 routes

• docosanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• octadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• octanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by NAGS, CROT, ACOT7mitochondrial matrix, cytosolOne of 2 routes

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamineReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. fatty acylamide amidohydrolase

  Catalysed by FAAHcytosol, extracellular space

• imidazole-4-acetaldehydeReach from here →
  1. transport of cysteine and histidine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)
  3. amine oxidase copper containing (histamine)

  Catalysed by SLC7A6, HDC, AOC2, AOC1 and 1 moreextracellular space, cytosolOne of 2 routes

• alpha-linolenic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• folic acidReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. Folate Transport by Oats

  Catalysed by CTH, SOD1, SLC22A8, SLC22A7cytosol, extracellular spaceOne of 4 routes

• selenomethionineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by CTH, AGXT2cytosol

• decanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosolOne of 2 routes

• CHEBI:27760Reach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by CTH, AGXT2cytosolOne of 2 routes

• 3,4-dihydroxyphenylacetaldehydeReach from here →
  1. cystine to cysteine conversion
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. monoamine oxidase (dopamine)

  Catalysed by SLCO1A2, SLCO1B3, AOC2, AOC1 and 3 morecytosol, extracellular spaceOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

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

• (2-hydroxyphenyl)acetic acidReach from here →
  1. transport of cysteine and phenylalanine (cytosol to extracellular)
  2. Phenylalanine Transaminase, Mitochondrial
  3. Phenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by SLC7A5, GOT2, TAT, HPDextracellular space, cytosol, mitochondrial matrix

• 2-oxopentanoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by CTH, AGXT2cytosol

• O-decanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosol

• 17alpha-hydroxypregnenoloneReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular space, endoplasmic reticulum

• CHEBI:28862Reach from here →
  1. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  2. glycyl-tRNA(gly) phosphorylation
  3. 2'-deoxyguanosine 5'-diphosphate:oxidized-thioredoxin 2'-oxidoreductase

  Catalysed by CARS1, TXN, RRM1, RRM2 and 2 morecytosol

• CHEBI:28918Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:phenylethanolamine N-methyltransferase

  Catalysed by SLCO1A2, SLCO1B3, PNMTcytosol, extracellular space

• a dicarboxylateReach from here →
  1. L-Cysteine:2-oxoglutarate aminotransferase
  2. 2-oxo-3-sulfanylpropanoate + L-kynurenine = kynurenate + L-cysteine + H2O
  3. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)

  Catalysed by GOT2, GOT1, AADAT, SLC22A8cytosol, inner side of the membrane, outer side of the membraneOne of 10 routes

• N-glycoloylneuraminateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CMP-N-acetylneuraminate monooxygenase (N-acetylneuraminate)

  Catalysed by CTH, NPLcytosol

• iron(2+)Reach from here →
  1. cystine to cysteine conversion
  2. glutathione:dehydroascorbate oxidoreductase
  3. cytochrome b reductase (ascorbate)

  Catalysed by CYBRD1cytosol, extracellular spaceOne of 2 routes

• Mn(2+)Reach from here →
  1. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  2. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)
  3. Mn(2+)(out) + Ca(2+)(in) = Mn(2+)(in) + Ca(2+)(out)

  Catalysed by SLC1A1, SLC24A1, SLC24A5, SLC24A4 and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• a carboxylateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by CTH, FAHD1cytosol

• L-ascorbic acidReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. L-dehydroascorbate + 2 glutathione = glutathione disulfide + L-ascorbate

  Catalysed by CTH, TSTD1, CLIC1, GSTO1 and 1 morecytosol

• Zn(2+)Reach from here →
  1. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  2. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)
  3. Zn(2+)(in) + Ca(2+)(out) = Zn(2+)(out) + Ca(2+)(in)

  Catalysed by SLC1A1, SLC24A1, SLC24A5, SLC24A4 and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

  Catalysed by SLC7A9, SLC3A1, BCAT2, BCKDHA and 1 moreextracellular space, cytosol, mitochondrial matrix

• Asp-tRNA(Asp)Reach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by CDO1, SLC25A12, SLC25A13, DARS1cytosol, mitochondrial matrix

• hypoioditeReach from here →
  1. transport of cysteine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by SLC7A5, TPO, MPO, LPOextracellular space, cytosol

• hydrogenselenideReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

  Catalysed by SLC7A5, MOCOS, SCLYextracellular space, cytosol

• phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by NAGS, CHAT, PHOSPHO1mitochondrial matrix, nucleusOne of 2 routes

• glycolateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, DAO and 1 moreextracellular space, cytosol, peroxisomeOne of 3 routes