L-cysteine

Three steps

• carnosineReach 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 + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC7A5, SLC7A10, CARNS1extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-oxoadipateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

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

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

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 2 routes

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular spaceOne of 4 routes

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glycerol-3-phosphate 1-O-acyltransferase (palmitoyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol

• a 1-acylglycerone 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by CDO1, SLC25A12, SLC25A13, ADSS2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 9 routes

• beta-D-fructose 6-phosphateReach from here →
  1. transport of cysteine and glutamine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. alpha-D-glucosamine 6-phosphate + H2O = beta-D-fructose 6-phosphate + NH4(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A10, GFPT2 and 3 moreextracellular space, cytosol

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

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

• succinate semialdehydeReach from here →
  1. transport of chloride and cysteine (cytosol to extracellular)
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate + 2-oxoglutarate = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A14, SLC6A12, ABATextracellular space, cytosol

• CMP-N-acetyl-beta-neuraminateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CTP:N-acetylneuraminate cytidylyltransferase

  Catalysed by CTH, NPL, CMAScytosol, nucleusOne of 2 routes

• N-sulfo-D-glucosamineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by CTH, SOD1, SGSHcytosol

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

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

• dehydroepiandrosterone 3-sulfateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by CTH, SOD1, STScytosol

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

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

• 2-hydroxy-3-oxopropanoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate aldose-ketose-isomerase

  Catalysed by CTH, AGXT, HYIcytosolOne of 2 routes

• dimethylamineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AGXT2 and 1 moreextracellular space, cytosolOne of 2 routes

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

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

• L-cysteateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by NAGS, BAAT, GADL1, CSADmitochondrial matrix, cytosol

• N(1)-acetylspermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

  Catalysed by NAGS, SAT1, SAT2, PAOXmitochondrial matrix, cytosol

• porphobilinogenReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by SLC3A2, SLC7A5, SLC7A8, ALAS1 and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• phosphoethanolamineReach from here →
  1. transport of cysteine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. phosphoethanolamine + H2O = acetaldehyde + NH4(+) + phosphate

  Catalysed by SLC7A5, ETNPPLextracellular space, cytosol

• adenosine 5'-phosphosulfateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. ATP:sulfate adenylyltransferase

  Catalysed by CTH, SOD1, PAPSS1, PAPSS2cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by SLC3A2, SLC7A5, SLC7A8, PIPOX and 1 moreextracellular space, cytosolOne of 3 routes

• N-acetylputrescineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetylputrescine + H2O = putrescine + acetate

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

• 4-aminobutanalReach from here →
  1. transport of arginine and cysteine (cytosol to extracellular)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by SLC7A6, SLC3A2, SLC7A7, AOC1extracellular space, cytosol

• L-glutamyl 5-phosphateReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate
  3. L-glutamate 5-semialdehyde + phosphate + NADP(+) = L-glutamyl 5-phosphate + NADPH + H(+)

  Catalysed by SLC7A6, OAT, ALDH18A1extracellular space, cytosol

• (2R)-2-phosphoglycerateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)

  Catalysed by CTH, PKM, PKLR, ENO1 and 2 morecytosol

• 3-oxosphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

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

• (S)-2-amino-6-oxohexanoateReach from here →
  1. transport of cysteine and lysine (cytosol to extracellular)
  2. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)
  3. L-saccharopine + NAD(+) + H2O = (S)-2-amino-6-oxohexanoate + L-glutamate + NADH + H(+)

  Catalysed by SLC7A6, AASSextracellular space, cytosol, mitochondrial matrix

• N(1)-acetylspermidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine + acetyl-CoA = N(1)-acetylspermidine + CoA + H(+)

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• 3-aminopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. spermidine:oxygen oxidoreductase (spermidine-forming)

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

• 5-oxo-L-prolineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by CTH, TSTD1, CHAC2, CHAC1cytosol

• L-2-aminopentanoateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AGXT2extracellular space, cytosolOne of 2 routes

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

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

• L-2-aminohexanoateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AGXT2extracellular space, cytosolOne of 2 routes

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SHMT1 and 1 moreextracellular space, cytosol

• N(8)-acetylspermidineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N(8)-acetylspermidine + H2O = spermidine + acetate

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular spaceOne of 4 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA hydrolase

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

• dCDPReach from here →
  1. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  2. glycyl-tRNA(gly) phosphorylation
  3. 2'-deoxycytidine diphosphate:oxidized-thioredoxin 2'-oxidoreductase

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

• N(tau)-methylhistamineReach from here →
  1. transport of cysteine and histidine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)
  3. S-Adenosyl-L-methionine:histamine N-tele-methyltransferase

  Catalysed by SLC7A6, HDC, HNMTextracellular space, cytosolOne of 3 routes

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

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 14 moremitochondrial matrix, Golgi apparatus

• (S)-3-amino-2-methylpropanoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-3-amino-isobutanoate:2-oxoglutarate aminotransferase

  Catalysed by CTH, AGXT2, ABATcytosol, mitochondrial matrix

• L-2-aminoadipateReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. 2-oxoadipate + L-kynurenine = L-2-aminoadipate + kynurenate + H2O

  Catalysed by SLC7A5, KYNU, AADATextracellular space, cytosol

• phosphoenolpyruvateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by CTH, PC, PCK1, PCK2cytosol, mitochondrial matrix

• an N-acetyl-L-cysteine-S-conjugateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate

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

• hyodeoxycholateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and lithocholate (cytosol to extracellular)
  3. cytochrome P450 (lithocholate)

  Catalysed by SLCO1A2, SLCO1B3, CYP3A4cytosol, extracellular space