L,L-cystathionine

Three steps

• iodideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by CTH, SLC7A5, TPOcytosol, extracellular spaceOne of 3 routes

• nitric oxideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by CTH, SLC7A6, NOS3, NOS1 and 1 morecytosol, extracellular spaceOne of 2 routes

• CHEBI:16523Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by CTH, SLC7A6, ACY1cytosol, extracellular space

• 2-phenylacetamideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by CTH, SLC7A5, EPXcytosol, extracellular space

• CHEBI:16633Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Selenocystathionine Lysteine-lyase (deaminating)
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by CTHcytosolOne of 2 routes

• Met-tRNA(Met)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, MARS1cytosol, extracellular space

• (S)-lactateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by CTH, AGXT2, LDHA, LDHB and 3 morecytosol

• CHEBI:16668Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by CTH, CDO1, GAD1, CSADcytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by CTH, AGXT2, AADATcytosolOne of 2 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by CTH, SLC7A5, CNDP2cytosol, extracellular space

• tryptamineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by CTH, SLC7A5, DDCcytosol, extracellular space

• 2-oxoglutaramateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by CTH, AGXT2, KYAT1cytosolOne of 5 routes

• formaldehydeReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2

  Catalysed by CTH, MOCOS, KDM1A, KDM1BcytosolOne of 3 routes

• 3-fumarylpyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by CTH, AGXT2, FAHD1cytosol

• pantetheine 4'-phosphateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by CTH, PPCS, PPCDCcytosolOne of 4 routes

• psychosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatus, cytosol

• glyoxylic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular space, peroxisome

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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

• bilirubin IXalphaReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• oxalic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by CTH, SLC6A14, SLC26A6cytosol, extracellular space

• a cholesterol esterReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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

• (R)-3-hydroxybutyric acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of (R)-3-hydroxybutanoate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 7 morecytosol, extracellular spaceOne of 4 routes

• taurolithocholateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

• 3-hydroxypyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by CTH, AGXT2, AGXTcytosol

• CHEBI:17208Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by CTHcytosol

• homocysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. Formation of Homocystine

  Catalysed by CTHcytosolOne of 2 routes

• L-glutamic 5-semialdehydeReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by CTH, SLC7A6, OATcytosol, extracellular space, mitochondrial matrix

• S-(4,8-dimethylnonanoyl)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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-cystathionine)
  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-cystathionine)
  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-cystathionine)
  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-cystathionine)
  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-cystathionine)
  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

• aceneuramateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by CTH, AGXT2, NPLcytosol

• 5'-deoxyadenosineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by CTH, AGXT2, TYW1B, TYW1cytosol

• all-trans-retinolReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by CTH, MOCOS, RETSATcytosolOne of 3 routes

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

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes

• CHEBI:17380Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by CTH, SLC7A5, PRODH2cytosol, extracellular spaceOne of 2 routes

• agmatineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by CTH, SLC7A6cytosol, extracellular space, mitochondrial matrix

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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

• AH2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by CTH, SLC7A5, MOCOScytosol, extracellular spaceOne of 26 routes

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

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

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

  Catalysed by NAGS, SAT1, SAT2, SRM and 1 moremitochondrial matrix, cytosolOne of 2 routes

• 5-aminolevulinic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 3 morecytosol, extracellular space, mitochondrial matrixOne of 2 routes

• all-trans-retinyl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  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, cytosol

• indole-3-pyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-tryptophan + glyoxylate = indole-3-pyruvate + glycine

  Catalysed by CTH, AGXT2, AADATcytosolOne of 3 routes

• Ala-tRNA(Ala)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, AARS1cytosol, extracellular space