(S)-methylmalonyl-CoA

Three steps

• D-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes

• an acylcholineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by CHAT, BCHEmitochondrial matrix, nucleus

• fatty acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 4 routes

• 5-aminolevulinateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by GLYAT, GLYATL2, GLYATL1, ALAS1 and 1 moremitochondrial matrixOne of 2 routes

• chenodeoxycholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. chenodeoxycholoyl-CoA hydrolase

  Catalysed by ACAA1, SCP2, MYO5B, ACOT7 and 2 moremitochondrial matrix, peroxisomeOne of 3 routes

• glycochenodeoxycholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, BAATmitochondrial matrix

• taurocholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. propanoyl-CoA C-acyltransferase
  3. Choloyl-CoA:glycine N-choloyltransferase

  Catalysed by ACAA1, SCP2, MYO5B, BAATmitochondrial matrix, peroxisomeOne of 5 routes

• taurodeoxycholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

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

• glycochenodeoxycholic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by ACAA1, SCP2, MYO5B, BAATmitochondrial matrix, peroxisomeOne of 5 routes

• cholesteryl hexadecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol, endoplasmic reticulum

• glyoxylateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by GLYAT, GLYATL2, GLYATL1, AADATmitochondrial matrixOne of 12 routes

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moremitochondrial matrix, Golgi apparatus, cytosol

• CHEBI:36882Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2, AUHmitochondrial matrix

• 3-hydroxyisovaleric acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by CPT1A, CPT1Bmitochondrial matrix, cytosol

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• sphingosine 1-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• glycolithocholic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by GLYAT, GLYATL2, GLYATL1, BAATmitochondrial matrix, cytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, GCATmitochondrial matrix

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• (S)-lactic acidReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Propanoyl-CoA hydrolase
  3. transport of L-lactate and propanoate (cytosol to extracellular)

  Catalysed by MLYCD, ACOT7, SLC16A4, SLC16A5 and 4 morecytosol, extracellular spaceOne of 2 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Production of Glutaryl Carnitine
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by CPT1A, CPT1C, CPT1B, SUGCTmitochondrial matrix, cytosolOne of 2 routes

• phosphoenolpyruvic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. ATP citrate lyase (citrate)
  3. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by ACLY, PCK1mitochondrial matrix, cytosolOne of 2 routes

• 13-cis-retinolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 2 moremitochondrial matrix, cytosol

• spermineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

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

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by CPT1A, CPT1C, CPT1B, SOAT2 and 1 moremitochondrial matrix, cytosol, endoplasmic reticulum

• L-ornithineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by BAAT, GATMmitochondrial matrix, cytosolOne of 15 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 ASAH1, ACER2, ACER1, ASAH2 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• a 1,3-diacylglycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moremitochondrial matrix, Golgi apparatus

• a 1,2-diacylglycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• gamma-Glu-AlaReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by GGT1, GGT5, GGTLC2, GGT6 and 2 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GLDC and 1 moremitochondrial matrix

• an N-acylsphing-4-enineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moremitochondrial matrix, Golgi apparatus

• N-tetracosanoylsphinganineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosol

• N-hexacosanoylsphinganineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosol

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moremitochondrial matrix, Golgi apparatus

• acetoacetyl-CoAReach from here →
  1. Methylmalonyl-CoA epimerase
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate

  Catalysed by MCEE, MMUT, OXCT1mitochondrial matrix

• (2E)-butenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 4 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by GLYAT, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosol

• (2E)-glutaconyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by CPT1A, CPT1C, CPT1B, ACOX1mitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• hexadecanoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by CPT1A, CPT1C, CPT1B, TECRmitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• D-alanineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by GLYAT, GLYATL2, GLYATL1, PIPOXmitochondrial matrixOne of 2 routes

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by SAT1, SAT2, SRMmitochondrial matrix, cytosol

• 7,8-dihydrofolateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by BAAT, MTO1mitochondrial matrix, cytosol

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by GLYAT, GLYATL2, GLYATL1, SHMT1 and 1 moremitochondrial matrix

• propane-1,3-diamineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by SAT1, SAT2, DHPSmitochondrial matrix, cytosol