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Reach

3-hydroxyisovaleryl-CoA

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

Inferred

Every route here is a hypothesis drawn from reference reactions (Rhea and Human-GEM), not a finding. Currency species such as water, ATP and NAD(H) never form a step. Evidence key

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

Does it reach a compound?

Two steps

4,8-dimethylnonanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal
Catalysed by CPT1A, CPT1B, CRAT, CROTcytosol, peroxisomeOne of 3 routes
tetradecanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal
Catalysed by CPT1A, CPT1B, ACAA1, HSD17B4 and 2 morecytosol, peroxisome
hexadecanedioyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Production of Hexadecanedioylcarnitine
Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 2 routes
pristanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. fatty-acid-CoA ligase
Catalysed by CPT1A, CPT1B, SLC27A2cytosolOne of 2 routes
undecanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. medium-chain acyl-CoA ligase (undecylic acid)
Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 4 morecytosolOne of 3 routes
(5Z)-tetradecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (physeteric acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 8 routes
(3E)-decenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial
Catalysed by CPT1A, CPT1B, ACADM, ECHS1 and 2 morecytosol, mitochondrial matrix
(2R,6)-dimethylheptanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)
Catalysed by CPT1A, CPT1B, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrix
(7Z)-hexadecenoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 8 routes
tricosanoyl-CoAReach from here →
1. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
2. long-chain-fatty-acid-CoA ligase (tricosanoic acid)
Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 4 routes

Three steps

N(omega)-methyl-L-arginineReach from here →
Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrixOne of 2 routes
AReach from here →

Page 3 of 12

Catalysed by CPT1A, CPT1B, NAGS, MOCOScytosol, mitochondrial matrix

(5E)-tetradecenoyl-(R)-carnitineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Carnitine O-Palmitoyltransferase
carnitine O-palmitoyltransferase ((9E)-tetradecenoyl-CoA)

Catalysed by CPT1A, CPT1B, CPT1CcytosolOne of 7 routes

S-butanoyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Butanoate:CoA ligase (AMP-forming)
butanoyl-CoA + H2O = S-butanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 7 morecytosol

S-octanoyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Octanoyl-CoA:L-carnitine O-octanoyltransferase
octanoyl-CoA + H2O = S-octanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, CROT, NUDT19 and 2 morecytosol, mitochondrial matrix

decanoyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
medium-chain acyl-CoA ligase (decanoic acid)
decanoyl-CoA + H2O = decanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 5 morecytosol

S-dodecanoyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (lauric acid)
dodecanoyl-CoA + H2O = S-dodecanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 8 morecytosol

tetradecanoyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
tetradecanoyl-CoA + H2O = tetradecanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 8 morecytosol

an acyl-4'-phosphopantetheineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
acyl-CoA:sphingosine N-acyltransferase
an acyl-CoA + H2O = an acyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by CPT1A, CPT1B, ASAH1, ACER2 and 5 morecytosol, Golgi apparatus

tauroursodeoxycholateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
taurine to tauroursodeoxycholate conversion

Catalysed by CPT1A, CPT1B, BAATcytosolOne of 2 routes

glycoursodeoxycholateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
glycine to glycoursodeoxycholate conversion

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

leukotriene F4Reach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
N-acetyl-LTE4 to leukotriene E4 conversion
leukotriene F4 hydrolysis

Catalysed by CPT1A, CPT1B, ZDHHC11B, NAA10 and 30 morecytosol

2,4-dinitrophenyl-S-glutathioneReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
N-acetyl-LTE4 to leukotriene E4 conversion
transport of leukotriene E4 (cytosol to extracellular)

Catalysed by CPT1A, CPT1B, ZDHHC11B, NAA10 and 31 morecytosol, extracellular spaceOne of 2 routes

N-(9Z-octadecenoyl)glycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
(9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix

N-octadecanoylglycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (stearate)
octadecanoyl-CoA + glycine = N-octadecanoylglycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosolOne of 2 routes

N-(9Z-hexadecenoyl)-glycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (palmitolate)
(9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosolOne of 2 routes

2-hydroxyoctanoyl-CoAReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Octanoyl-CoA:L-carnitine O-octanoyltransferase
octanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyoctanoyl-CoA + succinate + CO2

Catalysed by CPT1A, CPT1B, CROT, PHYHcytosol, mitochondrial matrix

2-hydroxydecanoyl-CoAReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
medium-chain acyl-CoA ligase (decanoic acid)
decanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxydecanoyl-CoA + succinate + CO2

Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 5 morecytosol

2-hydroxyeicosanoyl-CoAReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (eicosanoate)
eicosanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyeicosanoyl-CoA + succinate + CO2

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

2-hydroxydodecanoyl-CoAReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (lauric acid)
dodecanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxydodecanoyl-CoA + succinate + CO2

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

2-hydroxytetradecanoyl-CoAReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
tetradecanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxytetradecanoyl-CoA + succinate + CO2

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

11-cis-retinyl tetradecanoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
11-cis-retinol + tetradecanoyl-CoA = 11-cis-retinyl tetradecanoate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosolOne of 2 routes

9-cis-retinyl tetradecanoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
9-cis-retinol + tetradecanoyl-CoA = 9-cis-retinyl tetradecanoate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

13-cis-retinyl tetradecanoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
13-cis-retinol + tetradecanoyl-CoA = 13-cis-retinyl tetradecanoate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

all-trans-retinyl tetradecanoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
all-trans-retinol + tetradecanoyl-CoA = all-trans-retinyl tetradecanoate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

tetradecyl tetradecanoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (myristic acid)
tetradecan-1-ol + tetradecanoyl-CoA = tetradecyl tetradecanoate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

1-(9Z-octadecenoyl)-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (DHA)
(4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z-octadecenoyl)-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphate + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

N-hexadecanoylglycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix

N-dodecanoylglycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (lauric acid)
dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosolOne of 2 routes

N-decanoylglycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
medium-chain acyl-CoA ligase (decanoic acid)
decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, ACSM4, ACSM1 and 5 morecytosolOne of 2 routes

N-octanoylglycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Octanoyl-CoA:L-carnitine O-octanoyltransferase
octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, CROT, GLYATL2cytosol, mitochondrial matrixOne of 2 routes

N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix

L-cystine-bis-glycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 3 morecytosol, mitochondrial matrix

an N-octadecanoyl-sphingoid baseReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase (stearate)
a sphingoid base + octadecanoyl-CoA = an N-octadecanoyl-sphingoid base + CoA + H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 6 morecytosol

glycyldehydrophenylalanineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 2 morecytosol, mitochondrial matrix

an N-(fatty acyl)-glycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
glycine N-acyltransferase (N-arachidonoylglycine)
a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrix

1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + CoA

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosol

N-(9Z,12Z-octadecadienoyl)-glycineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
(9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

Catalysed by CPT1A, CPT1B, SLC27A2, ACSL4 and 7 morecytosolOne of 2 routes

cholesteryl (7Z)-octadecenoateReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
(7Z)-octadecenoyl-CoA + cholesterol = cholesteryl (7Z)-octadecenoate + CoA

Catalysed by CPT1A, CPT1B, CPT1C, SOAT1cytosol

acetylcholineReach from here →

(3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
Acetyl-CoA:choline O-acetyltransferase
Acetyl-CoA:choline O-acetyltransferase

Catalysed by CPT1A, CPT1B, CHATcytosol, nucleusOne of 3 routes