(R)-methylmalonyl-CoA

Three steps

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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,12Z,15Z,18Z,21Z)-THA)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

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

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

• (17Z)-hexacosenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes

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

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

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

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. long-chain-fatty-acid-CoA ligase (DHA)

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

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

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

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

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

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

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

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

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

• (11Z)-octadecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• dodecanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Activation of dodecanedioic acid

  Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Production of Sebacoylcarnitine

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

• octanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Production of Suberylcarnitine

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

• hexanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

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

• (4Z,7Z,10Z,13Z,16Z)-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 ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

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

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

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by MMUT, ALAS1, CPNE7, CPT1A and 2 moremitochondrial matrix, cytosol

• (13Z)-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 ((13Z)-eicosenoic acid)

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by MMUT, ALAS1, CPNE7, CRAT and 1 moremitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by MMUT, ALAS1, CPNE7, ACAA1 and 3 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Production of Hexadecanedioylcarnitine

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

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

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by MMUT, ALAS1, CPNE7, ACSM4 and 5 moremitochondrial matrix, cytosolOne of 3 routes

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

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

• (3E)-decenoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by MMUT, ALAS1, CPNE7, ACADM and 3 moremitochondrial matrix

• O-3-oxobutanoyl-(R)-carnitineReach from here →
  1. (R)-methylmalonyl-CoA = succinyl-CoA
  2. acetoacetate + succinyl-CoA = acetoacetyl-CoA + succinate
  3. acetoacetyl-CoA + (R)-carnitine = O-3-oxobutanoyl-(R)-carnitine + CoA

  Catalysed by MMUT, OXCT1, CRAT

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
  2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by MMUT, ALAS1, CPNE7, HADHA and 2 moremitochondrial matrix

• (7Z)-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 (7-palmitoleic acid)

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

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

  Catalysed by MMUT, ALAS1, CPNE7, SLC27A2 and 7 moremitochondrial matrix, cytosolOne of 4 routes