(S)-methylmalonyl-CoA

Three steps

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by HADHA, ACADVL, HADHBmitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by HADHA, ACADVL, HADHB, ACADL and 1 moremitochondrial matrix

• (2E,4Z,7Z)-decatrienoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial
  3. Fatty Acid Beta Oxidation (C10:2->C10:3), Mitochondrial

  Catalysed by ECHS1, ACAA2, HADH, ACADMmitochondrial matrixOne of 3 routes

• docosanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• CHEBI:240943Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• octadecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• octanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by CROT, ACOT7mitochondrial matrix, cytosolOne of 2 routes

• alpha-linolenic acidReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Utilized Transport

  Catalysed by MLYCDcytosol, mitochondrial matrix, endoplasmic reticulumOne of 2 routes

• phenylacetylglycineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1mitochondrial matrix, cytosolOne of 6 routes

• decanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 3 moremitochondrial matrix, cytosolOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

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

• CHEBI:28666Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetyl-LTE4 to leukotriene E4 conversion
  3. LTD4 hydrolysis

  Catalysed by ZDHHC11B, NAA10, ESCO2, ESCO1 and 28 moremitochondrial matrix, cytosolOne of 2 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 4 moremitochondrial matrix, cytosol

• pyridoxine 5'-phosphateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SCLYmitochondrial matrix, cytosol

• N-acetyl-L-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by NAGSmitochondrial matrix

• K(+)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by NAGS, SLC1A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• Cys-tRNA(Cys)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by NAGS, CARS1mitochondrial matrix, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GARS1mitochondrial matrix, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-asparagine)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by NAGS, NARS1mitochondrial matrix, cytosol

• chlorine(.)Reach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Carnitine Transport by Atb0

  Catalysed by MLYCD, SLC6A14cytosol, mitochondrial matrix, extracellular spaceOne of 15 routes

• phosphocholineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by CHAT, PHOSPHO1mitochondrial matrix, nucleusOne of 2 routes

• glycocholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. propanoyl-CoA C-acyltransferase
  3. bile acid-CoA: amino acid N-acyltransferase

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

• cholateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. propanoyl-CoA C-acyltransferase
  3. choloyl-CoA hydrolase

  Catalysed by ACAA1, SCP2, MYO5B, ACOT8mitochondrial matrix, peroxisome, cytosolOne of 2 routes

• hydrogen sulfideReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by NAGS, CTHmitochondrial matrix, cytosolOne of 2 routes

• L-aspartateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by AGXT2mitochondrial matrixOne of 12 routes

• acetateReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Propanoyl-CoA hydrolase
  3. transport of acetate and propanoate (cytosol to extracellular)

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

• (9Z,12Z)-octadecadienoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosol

• 3-acetamidopropanalReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by PAOX, SMOXmitochondrial matrix, cytosolOne of 4 routes

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

  Catalysed by MLYCD, ACOT7, SLC16A4, SLC16A5 and 1 morecytosol, extracellular spaceOne of 2 routes

• citric acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. ATP citrate lyase (citrate)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by ACLY, CSmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by MLYCD, ACOT7, SLC16A4, SLC16A5 and 2 morecytosol, extracellular spaceOne of 3 routes

• tetradecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. Myristoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Utilized Transport

  Catalysed by MLYCDcytosol, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• adipic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• glutarateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + H2O = glutarate + CoA + H(+)

  Catalysed by CPT1A, CPT1C, CPT1B, ACOT8 and 1 moremitochondrial matrix, cytosolOne of 5 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by ACLY, GOT2mitochondrial matrix, cytosol

• docosan-1-olReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

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

• hexacosanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. Hexacosanoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• tetracosanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. Tetracosenoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• an N-acylsphinganineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

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

• octadecan-1-olReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

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

• eicosanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. Eicosanoyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoateReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Utilized Transport

  Catalysed by MLYCDcytosol, mitochondrial matrix, endoplasmic reticulumOne of 8 routes

• (15Z)-tetracosenoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. Utilized Transport

  Catalysed by MLYCDcytosol, mitochondrial matrix, endoplasmic reticulumOne of 4 routes

• (11Z)-eicosenoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

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

• putrescineReach 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, cytosolOne of 3 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by NAGS, IL4I1mitochondrial matrix, cytosol

• di-mu-sulfido-diiron(2+)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. De novo synthesis of 2Fe2S

  Catalysed by NAGS, NDUFAB1, FDXR, FXN and 4 moremitochondrial matrix