L,L-cystathionine

Three steps

• N-octanoyl-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-octanoyl-(2S)-hydroxyglycine = octanamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• N-decanoyl-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• N-dodecanoyl-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-dodecanoyl-(2S)-hydroxyglycine = dodecanamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• N-tetradecanoyl-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-tetradecanoyl-(2S)-hydroxyglycine = tetradecamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• N-(9Z-octadecenoyl)-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-(9Z-octadecenoyl)-(2S)-hydroxyglycine = (9Z)-octadecenamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycine = (9Z,12Z,15Z)-octadecatrienamide + glyoxylate

  Catalysed by CTH, AGXT2, PAMcytosol

• D-histidineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-histidine(in) + L-ornithine(out) + H(+)(in) = D-histidine(out) + L-ornithine(in) + H(+)(out)

  Catalysed by CTH, SLC7A6, SLC25A2cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-homoarginineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-homoarginine(in) + L-arginine(out) = L-homoarginine(out) + L-arginine(in)

  Catalysed by CTH, SLC7A6, SLC25A29cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and histidine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by CTH, SLC7A6, CARNS1cytosol, extracellular space

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

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

• L-cystine-bis-glycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by CTH, TXNRD1, TXNRD3, TXNRD2 and 2 morecytosolOne of 2 routes

• an N-octadecanoyl-sphingoid baseReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a sphingoid base + octadecanoyl-CoA = an N-octadecanoyl-sphingoid base + CoA + H(+)

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

• glycyldehydrophenylalanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

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

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 1 morecytosol, extracellular space

• kinetensinReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by CTH, SLC7A5, CMA1cytosol, extracellular spaceOne of 3 routes

• an N-(fatty acyl)-glycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

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

• 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + CoA

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

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

• cholesteryl (7Z)-octadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. (7Z)-octadecenoyl-CoA + cholesterol = cholesteryl (7Z)-octadecenoate + CoA

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

• acetaldehydeReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-threonine = 2-oxobutanoate + NH4(+)
  3. L-threonine = acetaldehyde + glycine

  Catalysed by CTH, SDS, SDSL, SHMT1cytosolOne of 2 routes

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

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 5 routes

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

  Catalysed by CTH, AGXT2, FAHD1cytosol

• all-trans-retinoic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 4 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by CTH, SLC7A5, TPH1, TPH2cytosol, extracellular spaceOne of 3 routes

• CHEBI:15467Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Acetoacetate:CoA ligase (AMP-forming)
  3. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)

  Catalysed by NAGS, AACS, HMGCS1mitochondrial matrix, cytosolOne of 3 routes

• CHEBI:15570Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 5 routes

• (S)-malateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

  Catalysed by CTH, AGXT2, CLYBLcytosolOne of 8 routes

• CHEBI:15611Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach from here →
  1. L-homocysteine + L-serine = L,L-cystathionine + H2O
  2. (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by CBS, MTR, SHMT1, SHMT2One of 2 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. (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

  Catalysed by CTH, SLC7A5, TPH1, TPH2cytosol, extracellular spaceOne of 2 routes

• betaine aldehydeReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. choline + A = betaine aldehyde + AH2

  Catalysed by CTH, MOCOS, CHDHcytosolOne of 4 routes

• trimethylenediamineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by CTH, SLC7A6, DHPScytosol, extracellular spaceOne of 3 routes

• hexadecanoic 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 4 routes

• CHEBI:15765Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by CTH, SLC7A5, THcytosol, extracellular spaceOne of 5 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

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

• bromideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by CTH, MGST3, GSTA4, GSTA1 and 14 morecytosolOne of 2 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 2 morecytosol, extracellular space

• thioredoxin dithiolReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by CTH, CARS1cytosolOne of 2 routes

• methanethiolReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by CTH, SELENBP1cytosol

• phosphatidylethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

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

• 11-cis-retinalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol:NAD+ oxidoreductase

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

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

• hexadecan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. formate dehydrogenase (palmitoyl-CoA)

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

• sulfateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. hydrogen sulfide + 2 O2 = sulfate + H(+)

  Catalysed by CTH, SOD1cytosolOne of 2 routes

• ureaReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by CTH, SLC7A6, ARG1cytosol, extracellular spaceOne of 3 routes

• 11-cis-retinyl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosolOne of 3 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CTH, AGXT2cytosol, mitochondrial matrix

• (R)-3-aminoisobutyric acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CTH, SLC7A5, AGXT2cytosol, extracellular space, mitochondrial matrix

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

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

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

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 10 moremitochondrial matrix, Golgi apparatusOne of 7 routes