L,L-cystathionine

Three steps

• octadecan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

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

• eicosanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. Eicosanoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoateReach 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 8 routes

• (15Z)-tetracosenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by CTHcytosol, extracellular spaceOne of 6 routes

• (11Z)-eicosenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

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

• L-lysineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by CTH, SLC7A9, SLC3A1, SLC7A7cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 5 routes

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

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

• putrescineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by CTH, SLC7A6, SLC3A2, SLC7A7cytosol, extracellular spaceOne of 7 routes

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

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

• 3-oxopropanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by CTH, AGXT2cytosol

• thiosulfateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate:sulfide sulfurtransferase

  Catalysed by CTH, GOT2, GOT1, MPST and 1 morecytosolOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by CTH, SLC7A5, IL4I1cytosol, extracellular space

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by CTH, NDUFAB1, FDXR, FXN and 10 morecytosol, mitochondrial matrix

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

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by CTH, SLC7A5, BCAT2, BCAT1cytosol, extracellular space

• 2-oxohexanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by CTH, AGXT2cytosol

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

  Catalysed by CTH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. D-serine = pyruvate + NH4(+)

  Catalysed by CTH, AGXT2, SRRcytosolOne of 11 routes

• an acylcholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by NAGS, CHAT, BCHEmitochondrial matrix, nucleus

• fatty acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• 5-aminolevulinateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 2 morecytosol, extracellular space

• 3-methyl-2-oxovaleric acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by CTH, SLC7A5, BCAT1cytosol, extracellular spaceOne of 2 routes

• chenodeoxycholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. chenodeoxycholoyl-CoA hydrolase

  Catalysed by NAGS, ACAA1, SCP2, MYO5B and 3 moremitochondrial matrix, peroxisomeOne of 3 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by CTH, AGXT2, AADATcytosolOne of 4 routes

• glycochenodeoxycholateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

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

• taurocholateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (GSH)

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 7 routes

• taurodeoxycholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by NAGS, SLC27A2, SLC27A5, BAATmitochondrial matrix, cytosolOne of 4 routes

• glycochenodeoxycholic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

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

• cholesteryl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

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

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

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

• CHEBI:36882Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2 and 1 moremitochondrial matrix

• 3-hydroxyisovaleric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by NAGS, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• sphingosine 1-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• glycolithocholic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

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

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• (R)-lactic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by CTH, AGXT2, LDHDcytosol

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

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 8 morecytosol, extracellular spaceOne of 7 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Glutaryl Carnitine
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

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

• 2-iminopropanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by CTH, AGXT2, RIDAcytosol

• phosphoenolpyruvic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by CTH, AGXT2, PKM, PKLRcytosolOne of 3 routes

• 13-cis-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 3 moremitochondrial matrix, cytosol

• spermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

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

• N-acetyl-beta-neuraminic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by CTH, AGXT2, NPLcytosol

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 2 moremitochondrial matrix, cytosol, endoplasmic reticulum

• N-(acetyl)-sphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

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

• a 1,3-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

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

• a 1,2-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatusOne of 2 routes