(R)-methylmalonyl-CoA

Three steps

• glutarateReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutaryl-CoA + H2O = glutarate + CoA + H(+)

  Catalysed by MMUT, SUGCT, ACOT8, ACOT4One of 5 routes

• (R)-mevalonateReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. (R)-mevalonate + 2 NADP(+) + CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + 2 NADPH + 2 H(+)

  Catalysed by MMUT, SUGCT, HMGCR

• CHEBI:36882Reach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by MMUT, SUGCT, AUHmitochondrial matrix

• taurineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by MMUT, ALAS1, CPNE7, BAATmitochondrial matrix, cytosolOne of 2 routes

• glycineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by MMUT, ALAS1, CPNE7, GLYAT and 2 moremitochondrial matrixOne of 10 routes

• (3S)-3-hydroxybutanoyl-CoAReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate
  3. (3S)-3-hydroxybutanoyl-CoA + NAD(+) = acetoacetyl-CoA + NADH + H(+)

  Catalysed by MMUT, OXCT1, HADH, HSD17B10

• a (3S)-3-hydroxyacyl-CoAReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. a 3-oxo acid + succinyl-CoA = a 3-oxoacyl-CoA + succinate
  3. a (3S)-3-hydroxyacyl-CoA + NAD(+) = a 3-oxoacyl-CoA + NADH + H(+)

  Catalysed by MMUT, OXCT1, OXCT2, EHHADH and 2 more

• (S)-methylmalonyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by MMUT, ALAS1, CPNE7, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)

  Catalysed by MMUT, SUGCT, GCDHmitochondrial matrixOne of 2 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by MMUT, ALAS1, CPNE7, ACAA1 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• (2E)-glutaconyl-CoAReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. glutarate + succinyl-CoA = glutaryl-CoA + succinate
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by MMUT, SUGCT, ACOX1

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by MMUT, ALAS1, CPNE7, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• eicosanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 12 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by MMUT, ALAS1, CPNE7, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. phytanate-CoA ligase

  Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 6 moremitochondrial matrix, cytosolOne of 15 routes

• sphing-4-enineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by MMUT, ALAS1, CPNE7, ASAH1 and 3 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by MMUT, ALAS1, CPNE7, SAT1 and 1 moremitochondrial matrix, cytosolOne of 3 routes

• L-methionineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. N-acetylglutamate synthase (N-acetylmethionine)

  Catalysed by MMUT, ALAS1, CPNE7, NAGSmitochondrial matrix

• L-alanineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

  Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrix

• L-asparagineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. N-acetylglutamate synthase (N-acetyl-L-asparagine)

  Catalysed by MMUT, ALAS1, CPNE7, NAGSmitochondrial matrix

• porphobilinogenReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by MMUT, ALAS1, ALAS2, CPNE7 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• L,L-cystathionineReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by MMUT, ALAS1, CPNE7, NAGSmitochondrial matrix

• an acyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by MMUT, ALAS1, CPNE7, ASAH1 and 3 moremitochondrial matrix, Golgi apparatusOne of 3 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by MMUT, ALAS1, CPNE7, HADHA and 2 moremitochondrial matrixOne of 3 routes

• (3S)-citramalyl-CoAReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. itaconate + succinyl-CoA = itaconyl-CoA + succinate
  3. (3S)-citramalyl-CoA = itaconyl-CoA + H2O

  Catalysed by MMUT, SUGCT, AUH

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 1 moremitochondrial matrix, endoplasmic reticulumOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 1 moremitochondrial matrix, cytosolOne of 4 routes

• decanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by MMUT, ALAS1, CPNE7, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by MMUT, ALAS1, CPNE7, CPT1A and 2 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 11 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by MMUT, ALAS1, CPNE7, CPT1A and 1 moremitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by MMUT, ALAS1, CPNE7, CPT1A and 2 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 5 routes