3-hydroxyisovaleryl-CoA

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

555 compounds within 3 steps: 2 in 1, 108 in 2, 445 in 3

Two steps

itaconyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Itaconate:CoA ligase (GDP-forming)
Catalysed by CPT1A, CPT1B, SUCLG1, SUCLG2 and 1 morecytosol, mitochondrial matrixOne of 2 routes
(9Z,12Z)-octadecadienoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 13 routes
tetradecanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (myristic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes
octanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
Catalysed by CPT1A, CPT1B, CROTcytosol, mitochondrial matrixOne of 20 routes
phytanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. phytanate-CoA ligase
Catalysed by CPT1A, CPT1BcytosolOne of 5 routes
octadecanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (stearate)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 5 morecytosolOne of 15 routes
sphing-4-enineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. acyl-CoA:sphingosine N-acyltransferase
Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 2 morecytosol, Golgi apparatusOne of 2 routes
spermidineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. diamine N-acetyltransferase (N1-acetylspermidine)
Catalysed by CPT1A, CPT1B, SAT1, SAT2cytosolOne of 3 routes
L-methionineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. N-acetylglutamate synthase (N-acetylmethionine)
Catalysed by CPT1A, CPT1B, NAGScytosol, mitochondrial matrix
L-alanineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
Catalysed by CPT1A, CPT1Bcytosol, mitochondrial matrix
L-asparagineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. N-acetylglutamate synthase (N-acetyl-L-asparagine)
Catalysed by CPT1A, CPT1B, NAGScytosol, mitochondrial matrix
L,L-cystathionineReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Catalysed by CPT1A, CPT1B, NAGScytosol, mitochondrial matrix
an acyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. acyl-CoA:sphingosine N-acyltransferase
Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 2 morecytosol, Golgi apparatusOne of 2 routes
(3Z)-dodecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)
Catalysed by CPT1A, CPT1B, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)
Catalysed by CPT1A, CPT1B, SLC27A2, SLC27A5cytosol, endoplasmic reticulumOne of 3 routes
deoxycholoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
Catalysed by CPT1A, CPT1B, SLC27A2, SLC27A5cytosolOne of 4 routes
decanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. medium-chain acyl-CoA ligase (decanoic acid)
Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 4 morecytosolOne of 7 routes
(2E)-hexadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Carnitine O-Palmitoyltransferase
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 4 routes
(9Z)-hexadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (palmitolate)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes
CHEBI:63544Reach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (adrenic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes
hexacosanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (cerotic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
tetracosanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (lignocerate)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 10 routes
docosanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (behenic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 9 routes
(2E)-octadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Carnitine O-Palmitoyltransferase
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 3 routes
(6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (stearidonic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 8 routes
(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (EPA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 9 routes
(7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (DPA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 6 routes
(9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (linolenate)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes
(13Z)-docosenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (cis-erucic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 6 routes
(11Z)-eicosenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes
(9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 4 routes
(15Z)-tetracosenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (nervonic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 6 routes
(17Z)-hexacosenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (ximenic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 4 routes
(8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
(8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
(4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (DHA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes
heptadecanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (margaric acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 6 routes
(9Z)-heptadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
(11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
nonadecanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (nonadecylic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 6 routes
(11Z)-octadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 3 routes
dodecanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Activation of dodecanedioic acid
Catalysed by CPT1A, CPT1BcytosolOne of 4 routes
decanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Production of Sebacoylcarnitine
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 4 routes
octanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Production of Suberylcarnitine
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 4 routes
hexanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Activation of adipic acid for Formation of Adipoyl Carnitine
Catalysed by CPT1A, CPT1BcytosolOne of 4 routes
(4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes
(11Z,14Z)-eicosadienoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes
(R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Production of 3-Hydroxyoctadecenoylcarnitinec
Catalysed by CPT1A, CPT1B, CPT1Ccytosol
(13Z)-eicosenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes