3-methyl-(2E)-glutaconyl-CoA

1. Formation of 3-Methyl-Glutaconate
2. Production of Suberylcarnitine

Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

1. Formation of 3-Methyl-Glutaconate
2. Activation of adipic acid for Formation of Adipoyl Carnitine

No catalyst recordedmitochondrial matrix, cytosolOne of 4 routes

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

1. Formation of 3-Methyl-Glutaconate
2. Production of 3-Hydroxyoctadecenoylcarnitinec

Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

Catalysed by SLC27A2, ACSL4, ACSL3, ACSL1 and 4 moremitochondrial matrix, cytosolOne of 5 routes

1. Formation of 3-Methyl-Glutaconate
2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

Catalysed by CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

1. Formation of 3-Methyl-Glutaconate
2. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

Catalysed by ACAA1, HSD17B4, EHHADH, ACOX1mitochondrial matrix, peroxisome

1. Formation of 3-Methyl-Glutaconate
2. Production of Hexadecanedioylcarnitine

Catalysed by CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

1. Formation of 3-Methyl-Glutaconate
2. fatty-acid-CoA ligase

Catalysed by SLC27A2mitochondrial matrix, cytosolOne of 2 routes

1. Formation of 3-Methyl-Glutaconate
2. medium-chain acyl-CoA ligase (undecylic acid)

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

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase (physeteric acid)

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

1. Formation of 3-Methyl-Glutaconate
2. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

Catalysed by ACADM, ECHS1, ACAA2, HADHmitochondrial matrix

1. Formation of 3-Methyl-Glutaconate
2. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

Catalysed by HADHA, ACAA2, HADHBmitochondrial matrix

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

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

1. Formation of 3-Methyl-Glutaconate
2. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

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

1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
2. hydroxymethylglutaryl-CoA lyase
3. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate

Catalysed by AUH, HMGCL, ADHFE1mitochondrial matrix, peroxisome

1. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)
2. hydroxymethylglutaryl-CoA lyase
3. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)

Catalysed by and 2 more