(S)-methylmalonyl-CoA

Three steps

• L-isoleucineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and isoleucine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 18 routes

• CHEBI:17203Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and proline (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 15 routes

• succinyl-4'-phosphopantetheineReach from here →
  1. Methylmalonyl-CoA epimerase
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. succinyl-CoA + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by MCEE, MMUT, NUDT19, NUDT7 and 1 moremitochondrial matrix

• S-(4,8-dimethylnonanoyl)-4'-phosphopantetheineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 CRAT, CROT, NUDT19mitochondrial matrix, peroxisome

• S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• S-(9Z-hexadecenoyl)-4'-phosphopantetheineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  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 SLC27A2, ACSL4, ACSL3, ACSL1 and 5 moremitochondrial matrix, cytosol

• itaconateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Itaconate:CoA ligase (GDP-forming)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 4 routes

• N-octadecanoyl-heptadecasphing-4-enineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. heptadecasphing-4-enine + octadecanoyl-CoA = N-octadecanoyl-heptadecasphing-4-enine + CoA + H(+)

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• all-trans-retinolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. all-trans-Retinol 11-cis-trans-isomerase

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 1 moremitochondrial matrix, cytosolOne of 2 routes

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

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

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

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

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by MLYCD, CPT2, CPT1Acytosol, mitochondrial matrix, peroxisomeOne of 14 routes

• AH2Reach 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, cytosolOne of 6 routes

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. Lecithin Retinol Acyltransferase (11-Cis)

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 1 moremitochondrial matrix, cytosol

• S-methyl-5'-thioadenosineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosylmethioninamine:spermidine 3-aminopropyltransferase

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

• 5-aminolevulinic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

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

• all-trans-retinyl hexadecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. all-trans-retinyl hexadecanoate + H2O = 11-cis-retinol + hexadecanoate + H(+)

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

• Ala-tRNA(Ala)Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AARS1mitochondrial matrix, cytosol

• CHEBI:17769Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine:(acceptor) oxidoreductase

  Catalysed by SAT1, SAT2mitochondrial matrix, cytosol

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

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

• a triacylglycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• CHEBI:17895Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and tyrosine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 19 routes

• butanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 8 moremitochondrial matrix, cytosol

• CHEBI:18019Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glycine and lysine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 10 routes

• L-glutamineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. transport of glutamine and glycine (cytosol to extracellular)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 29 routes

• spermine monoaldehydeReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by SAT1, SAT2, AOC2, AOC1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• CHEBI:18095Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of Hydroxy-Proline

  Catalysed by SLC1A4mitochondrial matrix, extracellular space, cytosol

• O-octanoyl-(R)-carnitineReach from here →
  1. methylmalonyl-CoA decarboxylase
  2. Active Transport
  3. solute carrier (L-carnitine)

  Catalysed by MLYCD, SLC25A20cytosol, mitochondrial matrixOne of 11 routes

• CHEBI:18132Reach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by CHAT, CHKA, CHKBmitochondrial matrix, nucleus, cytosol

• dodecanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

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

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by SAT1, SAT2, AOC2, AOC1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

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

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosol

• ophthalmateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSSmitochondrial matrix

• S-methylmercury-L-cysteineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by NAGS, SLC7A8mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

• 11-cis-retinyl esterReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 1 moremitochondrial matrix, cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. ATP citrate lyase (citrate)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by ACLY, AGXT2mitochondrial matrix, cytosol

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by GLYAT, GLYATL2, GLYATL1, AGXT2mitochondrial matrixOne of 2 routes

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CROT, MOGAT2, MOGAT1mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, SHMT1mitochondrial matrix, cytosolOne of 2 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by ACSM4, ACSM1, ACSM2A, ACSM3 and 3 moremitochondrial matrix, cytosol

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

  Catalysed by NAGS, CARS2mitochondrial matrix

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by CPT1A, CPT1C, CPT1B, ELOVL7 and 6 moremitochondrial matrix, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 7 moremitochondrial matrix, cytosolOne of 2 routes

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

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