L-cysteine

Three steps

• trans-1,2-dithiane-4,5-diolReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by SLC7A5, SCLYextracellular space, cytosol

• creatineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GATM and 1 moreextracellular space, cytosolOne of 2 routes

• ferrocytochrome cReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-Lactate:NAD+ oxidoreductase
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

  Catalysed by CTH, LDHB, LDHAL6B, CYB5D1cytosol, mitochondrial matrixOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by CDO1, SLC25A12, SLC25A13, NAT8Lcytosol, mitochondrial matrixOne of 2 routes

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase

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

• a cholesterol esterReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + cholesterol = a cholesterol ester + CoA

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

• glycolaldehydeReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)

  Catalysed by CTH, AGXTcytosolOne of 2 routes

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

  Catalysed by CTH, NPLcytosolOne of 2 routes

• 2-aminoacrylic acidReach from here →
  1. transport of cysteine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. thyroid peroxidase (3,5-diiodo-L-tyrosine)

  Catalysed by SLC7A5, TPOextracellular space, cytosol

• (R)-lactaldehydeReach from here →
  1. cystine to cysteine conversion
  2. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)
  3. (R)-Lactaldehyde:NAD+ oxidoreductase (glutathione-formylating)

  Catalysed by GLO1, GLOD4, ADH1C, ADH1A and 5 morecytosol

• (S)-4-hydroxymandelateReach from here →
  1. transport of cysteine and tyrosine (cytosol to extracellular)
  2. L-tyrosine:2-oxoglutarate aminotransferase
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by SLC7A5, GOT2, GOT1, TAT and 1 moreextracellular space, cytosol

• S-(4,8-dimethylnonanoyl)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal
  3. 4,8-dimethylnonanoyl-CoA + H2O = S-(4,8-dimethylnonanoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by NAGS, CRAT, CROT, NUDT19mitochondrial matrix, peroxisome

• S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (linolenate)
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + H2O = S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• S-(9Z-hexadecenoyl)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. (9Z)-hexadecenoyl-CoA + H2O = S-(9Z-hexadecenoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

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

• itaconateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 4 routes

• N-octadecanoyl-heptadecasphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. heptadecasphing-4-enine + octadecanoyl-CoA = N-octadecanoyl-heptadecasphing-4-enine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol

• dehydroascorbic acidReach from here →
  1. cystine to cysteine conversion
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by SLCO1A2, SLCO1B3, DBHcytosol, extracellular spaceOne of 19 routes

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

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

• linoleic 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 3 routes

• sulfiteReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase
  3. 3-sulfinylpyruvic acid hydrolysis

  Catalysed by CDO1, GOT1cytosolOne of 17 routes

• D-aspartic acidReach from here →
  1. transport of chloride and cysteine (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC6A14, SLC12A2, SLC12A1, SLC1A7 and 4 moreextracellular space, cytosol

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

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

• enol-oxaloacetateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by CTH, ME2, SDHB, SDHAcytosol

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol, peroxisomeOne of 14 routes

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. Lecithin Retinol Acyltransferase (11-Cis)

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosolOne of 2 routes

• S-methyl-5'-thioadenosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosylmethioninamine:spermidine 3-aminopropyltransferase

  Catalysed by NAGS, SAT1, SAT2, SRM and 1 moremitochondrial matrix, cytosolOne of 4 routes

• diiodineReach from here →
  1. transport of cysteine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 2 routes

• all-trans-retinyl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. all-trans-retinyl hexadecanoate + H2O = 11-cis-retinol + hexadecanoate + H(+)

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 3 moremitochondrial matrix, cytosolOne of 3 routes

• nitrateReach from here →
  1. transport of arginine and cysteine (cytosol to extracellular)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by SLC7A6, NOS3, NOS1, NOS2 and 1 moreextracellular space, cytosol

• 3,3',5-triiodo-L-thyronine sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and triiodothyronine (cytosol to extracellular)
  3. aryl sulfotransferase (PAPS)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4 and 2 morecytosol, extracellular spaceOne of 2 routes

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

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4 and 1 morecytosol, extracellular space

• 3,3',5'-triiodo-L-thyronine sulfateReach 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'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by SLC7A5, MOCOS, DIO1extracellular space, cytosol

• 3,3'-diiodo-L-thyronineReach 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'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 5 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. Steryl-Sulfatase

  Catalysed by CTH, SOD1, STScytosol, Golgi apparatusOne of 4 routes

• N-acetylserotoninReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. acetyl-CoA:aralkylamine N-acetyltransferase

  Catalysed by SLCO1A2, SLCO1B3, AANATcytosol, extracellular space

• CHEBI:17724Reach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GNMT and 2 moreextracellular space, cytosolOne of 4 routes

• 4-methylumbelliferyl acetateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 5 morecytosol, extracellular space

• CHEBI:17769Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine:(acceptor) oxidoreductase

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• urateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by CTH, TSTD1, SLC22A13cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• prostaglandin F1Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin E1 (cytosol to extracellular)
  3. prostaglandin E1 + NADPH + H(+) = prostaglandin F1 + NADP(+)

  Catalysed by SLCO2A1, DHRS1cytosol, extracellular space

• a 1,2-diacyl-sn-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacyl-sn-glycerol + CoA

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

• methylarsonous acidReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GSTO1, GSTO2cytosol

• a triacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatus, cytosol

• all-trans-retinalReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinoate reduction

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular spaceOne of 7 routes

• 3-oxopropanoic acidReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A5, SLC7A10, ABATextracellular space, cytosol, inner side of the membrane, outer side of the membrane, mitochondrial matrix

• butanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 9 moremitochondrial matrix, cytosol

• fumaric acidReach from here →
  1. L-Cysteine:2-oxoglutarate aminotransferase
  2. 3-mercaptopyruvate:sulfide sulfurtransferase
  3. transport of fumarate and thiosulfate (cytosol to mitochondria)

  Catalysed by GOT2, GOT1, MPST, TST and 1 morecytosol, mitochondrial matrixOne of 12 routes

• 4-fumarylacetoacetateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by CTH, FAHcytosol, mitochondrial matrix

• a diacylglycerolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. a triacylglycerol + all-trans-retinol = an all-trans-retinyl ester + a diacylglycerol

  Catalysed by MOCOS, RETSAT, PNPLA4, PNPLA2