L,L-cystathionine
362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API
739 compounds within 3 steps: 4 in 1, 155 in 2, 580 in 3
Three steps
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- octadecanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-octadecanoyl-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- (9Z,12Z)-octadecadienoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- (9Z,12Z)-octadecadienoyl-CoA + 2 NADPH + 2 H(+) = (9Z,12Z)-octadecadien-1-ol + 2 NADP(+) + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (EPA)
- (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (DPA)
- (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoA + malonyl-CoA + H(+) = (9Z,12Z,15Z,18Z,21Z)-3-oxotetracosapentaenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (cerotic acid)
- hexacosanoyl-CoA + malonyl-CoA + H(+) = 3-oxooctacosanyol-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (behenic acid)
- very-long-chain 3-oxoacyl-CoA synthase (docosanoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 12 moremitochondrial matrix, cytosolOne of 3 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (lignocerate)
- very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 12 moremitochondrial matrix, cytosolOne of 3 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (palmitolate)
- 1-hexadecanoyl-sn-glycero-3-phosphate + (9Z)-hexadecenoyl-CoA = 1-hexadecanoyl-2-[(9Z)-hexadec-9-enoyl]-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (palmitolate)
- 1-hexadecanoyl-sn-glycero-3-phosphoethanolamine + (9Z)-hexadecenoyl-CoA = 1-hexadecanoyl-2-(9Z)-hexadecenoyl-sn-glycero-3-phosphoethanolamine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (palmitolate)
- (9Z)-hexadecenoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-(9Z-hexadecenoyl)-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- (9Z,12Z)-octadecadienoyl-CoA + malonyl-CoA + H(+) = (11Z,14Z)-3-oxoicosa-11,14-dienoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (linolenate)
- (9Z,12Z,15Z)-octadecatrienoyl-CoA + malonyl-CoA + H(+) = (11Z,14Z,17Z)-3-oxoeicosatrienoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
- (11Z)-eicosenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(13Z)-docosenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (cis-erucic acid)
- (13Z)-docosenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(15Z)-tetracosenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- octadecanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyoctadecanoyl-CoA + succinate + CO2
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (nervonic acid)
- (15Z)-tetracosenoyl-CoA + sphinganine = N-(15Z-tetracosenoyl)-sphinganine + CoA + H(+)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (lignocerate)
- heptadecasphing-4-enine + tetracosanoyl-CoA = N-tetracosanoyl-heptadecasphing-4-enine + CoA + H(+)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (cerotic acid)
- acyl-CoA oxidase (hexacosanoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, peroxisomeOne of 2 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (DHA)
- (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)
- (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z,17Z,20Z,23Z)-hexacosahexaenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- acyl-CoA:sphingosine N-acyltransferase
- N-(15Z-tetracosenoyl)-sphing-4-enine + H2O = (15Z)-tetracosenoate + sphing-4-enine
Catalysed by NAGS, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatus
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- acyl-CoA:sphingosine N-acyltransferase
- tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)
Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- glycerol-3-phosphate 1-O-acyltransferase (linoleoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 2 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate + octadecanoyl-CoA = 1-(9Z-octadecenoyl)-2-octadecanoyl-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (margaric acid)
- heptadecanoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z)-octadecenoyl-2-heptadecanoyl-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate + (9Z,12Z)-octadecadienoyl-CoA = 1-(9Z)-octadecenoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 11 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- glycerol-3-phosphate 1-O-acyltransferase (sn-glycerol-3-phosphate)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 2 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (myristic acid)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate + tetradecanoyl-CoA = 1-(9Z)-octadecenoyl-2-tetradecanoyl-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)
- (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(11Z,14Z,17Z,20Z,23Z)-hexacosapentaenoyl-CoA + CO2 + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + (9Z,12Z)-octadecadienoyl-CoA = 1-(9Z)-octadecenoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (eicosanoate)
- acyl-CoA oxidase (eicosanoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, peroxisomeOne of 4 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (behenic acid)
- acyl-CoA oxidase (docosanoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, peroxisomeOne of 4 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (lignocerate)
- acyl-CoA oxidase (tetracosanoyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, peroxisomeOne of 3 routes
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (palmitolate)
- (9Z)-hexadecenoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z-octadecenoyl)-2-(9Z-hexadecenoyl)-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-L-serine + (9Z,12Z)-octadecadienoyl-CoA = 1-(9Z-octadecenoyl)-2-(9Z,12Z-octadienoyl)-sn-glycero-3-phospho-L-serine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (palmitolate)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-L-serine + (9Z)-hexadecenoyl-CoA = 1-(9Z-octadecenoyl)-2-(9Z-hexadecenoyl)-sn-glycero-3-phospho-L-serine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-L-serine + octadecanoyl-CoA = 1-(9Z-octadecenoyl)-2-octadecanoyl-sn-glycero-3-phospho-L-serine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (eicosanoate)
- eicosanoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z)-octadecenoyl-2-eicosanoyl-sn-glycero-3-phosphate + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Arachidonate:CoA ligase (AMP-forming)
- glycerol-3-phosphate 1-O-acyltransferase (arachidonyl-CoA)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol
- 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholineReach from here →
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (DHA)
- (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- 1-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(9Z)-octadecenoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphoethanolamine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (lauric acid)
- dodecanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-dodecanoyl-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- 1-hexadecanoyl-sn-glycero-3-phospho-L-serine + (9Z,12Z)-octadecadienoyl-CoA = 1-hexadecanoyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho-L-serine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (stearate)
- 2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- a 1-acyl-sn-glycero-3-phospho-L-serine + (9Z,12Z)-octadecadienoyl-CoA = 1-acyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho-L-serine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
- a 1-acyl-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphoethanolamine + CoA
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol