CHEBI:15380
362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API
558 compounds within 3 steps: 3 in 1, 109 in 2, 446 in 3
Three steps
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetyl-LTE4 to leukotriene E4 conversion
- LTD4 hydrolysis
Catalysed by ALAS1, CPNE7, ZDHHC11B, NAA10 and 30 moremitochondrial matrix, cytosolOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- medium-chain acyl-CoA ligase (decanoic acid)
- decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA
Catalysed by ALAS1, CPNE7, ACSM4, ACSM1 and 6 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- amino-acid N-acetyltransferase (N-acetyl-L-alanine)
- L-selenocysteine selenide-lyase (L-alanine-forming)
Catalysed by ALAS1, CPNE7, SCLYmitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetyl-L-cysteine)
- K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
Catalysed by ALAS1, CPNE7, NAGS, SLC1A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetyl-L-cysteine)
- L-Cysteine:tRNA(Cys) ligase (AMP-forming)
Catalysed by ALAS1, CPNE7, NAGS, CARS1mitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- glycine N-acyltransferase (N-arachidonoylglycine)
- glycine:tRNA(Gly) ligase (AMP-forming)
Catalysed by ALAS1, CPNE7, GLYAT, GLYATL2 and 2 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetyl-L-asparagine)
- L-Asparagine:tRNA(Asn) ligase (AMP-forming)
Catalysed by ALAS1, CPNE7, NAGS, NARS1mitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Carnitine O-Palmitoyltransferase
- Carnitine Transport by Atb0
Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 2 moremitochondrial matrix, cytosol, extracellular spaceOne of 15 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Acetyl-CoA:choline O-acetyltransferase
- phosphocholine + H2O = choline + phosphate
Catalysed by ALAS1, CPNE7, CHAT, PHOSPHO1mitochondrial matrix, nucleusOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- propanoyl-CoA C-acyltransferase
- bile acid-CoA: amino acid N-acyltransferase
Catalysed by ALAS1, CPNE7, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- propanoyl-CoA C-acyltransferase
- choloyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisome, cytosolOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetyl-L-cysteine)
- L-cysteine hydrogen-sulfide-lyase (deaminating
Catalysed by ALAS1, CPNE7, NAGS, CTHmitochondrial matrix, cytosolOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- amino-acid N-acetyltransferase (N-acetyl-L-alanine)
- oxaloacetate + L-alanine = L-aspartate + pyruvate
Catalysed by ALAS1, CPNE7, AGXT2mitochondrial matrixOne of 12 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 10 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N1,N12-diacetylspermine to N1-acetylspermine conversion
- N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)
Catalysed by ALAS1, CPNE7, PAOX, SMOXmitochondrial matrix, cytosolOne of 4 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Propanoate:CoA ligase (AMP-forming)
- Propanoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, ACSS3, ACSS2 and 2 moremitochondrial matrix, cytosolOne of 3 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- ATP citrate lyase (citrate)
- acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
Catalysed by ALAS1, CPNE7, ACLY, CSmitochondrial matrix, cytosolOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Butanoate:CoA ligase (AMP-forming)
- Butyryl-CoA hydrolase
Catalysed by ALAS1, CPNE7, ACSM4, ACSM1 and 5 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (myristic acid)
- Myristoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 11 moremitochondrial matrix, cytosolOne of 2 routes
Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Activation of adipic acid for Formation of Adipoyl Carnitine
- Thioesterification of adipoyl-CoA for Release into Cytosol
Catalysed by ALAS1, CPNE7, ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Production of Glutaryl Carnitine
- glutaryl-CoA + H2O = glutarate + CoA + H(+)
Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 3 moremitochondrial matrix, cytosolOne of 5 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- ATP citrate lyase (citrate)
- D-aspartate:2-oxoglutarate aminotransferase
Catalysed by ALAS1, CPNE7, ACLY, GOT2mitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (behenic acid)
- docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (cerotic acid)
- Hexacosanoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosolOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (lignocerate)
- Tetracosenoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosolOne of 4 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- acyl-CoA:sphingosine N-acyltransferase
- sphingosine N-acyltransferase (sphinganine)
Catalysed by ALAS1, CPNE7, ASAH1, ACER2 and 8 moremitochondrial matrix, Golgi apparatus, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (stearate)
- octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 7 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (eicosanoate)
- Eicosanoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 9 moremitochondrial matrix, cytosolOne of 4 routes
Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 8 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (nervonic acid)
- (15Z)-tetracosenoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosol
Catalysed by ALAS1, CPNE7, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 4 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
- (11Z)-eicosenoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 9 moremitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- diamine N-acetyltransferase (N1-acetylspermidine)
- S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)
Catalysed by ALAS1, CPNE7, SAT1, SAT2 and 1 moremitochondrial matrix, cytosolOne of 3 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetylmethionine)
- L-Methionine:oxygen oxidoreductase (deaminating)
Catalysed by ALAS1, CPNE7, NAGS, IL4I1mitochondrial matrix, cytosol
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- N-acetylglutamate synthase (N-acetyl-L-cysteine)
- De novo synthesis of 2Fe2S
Catalysed by ALAS1, CPNE7, NAGS, NDUFAB1 and 6 moremitochondrial matrix
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- amino-acid N-acetyltransferase (N-acetyl-L-alanine)
- D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)
Catalysed by ALAS1, CPNE7, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- amino-acid N-acetyltransferase (N-acetyl-L-alanine)
- D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
Catalysed by ALAS1, CPNE7, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 6 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Acetyl-CoA:choline O-acetyltransferase
- an acylcholine + H2O = a carboxylate + choline + H(+)
Catalysed by ALAS1, CPNE7, CHAT, BCHEmitochondrial matrix, nucleus
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- long-chain-fatty-acid-CoA ligase (elaidate)
- (10Z)-heptadecenoic acid formation
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- glycine N-acyltransferase (N-arachidonoylglycine)
- succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA
Catalysed by ALAS1, CPNE7, GLYAT, GLYATL2 and 2 moremitochondrial matrixOne of 2 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Propanoyl-CoA:acetyl-CoA C-acyltransferase
- chenodeoxycholoyl-CoA hydrolase
Catalysed by ALAS1, CPNE7, ACAA1, SCP2 and 4 moremitochondrial matrix, peroxisomeOne of 3 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- glycine N-acyltransferase (N-arachidonoylglycine)
- chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)
Catalysed by ALAS1, CPNE7, GLYAT, GLYATL2 and 2 moremitochondrial matrix
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- propanoyl-CoA C-acyltransferase
- Choloyl-CoA:glycine N-choloyltransferase
Catalysed by ALAS1, CPNE7, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
- bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
Catalysed by ALAS1, CPNE7, SLC27A2, SLC27A5 and 1 moremitochondrial matrix, cytosolOne of 4 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Propanoyl-CoA:acetyl-CoA C-acyltransferase
- bile acid-CoA: amino acid N-acyltransferase
Catalysed by ALAS1, CPNE7, ACAA1, SCP2 and 2 moremitochondrial matrix, peroxisomeOne of 5 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- Palmitate:CoA ligase (AMP-forming)
- Acyl-CoA:cholesterol O-acyltransferase
Catalysed by ALAS1, CPNE7, SLC27A2, ACSL4 and 8 moremitochondrial matrix, cytosol, endoplasmic reticulum
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- glycine N-acyltransferase (N-arachidonoylglycine)
- glycine + 2-oxoglutarate = glyoxylate + L-glutamate
Catalysed by ALAS1, CPNE7, GLYAT, GLYATL2 and 2 moremitochondrial matrixOne of 11 routes
- 5-aminolevulinate synthase (2-amino-3-oxoadipate)
- acyl-CoA:sphingosine N-acyltransferase
- Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase
Catalysed by ALAS1, CPNE7, ASAH1, ACER2 and 6 moremitochondrial matrix, Golgi apparatus, cytosol