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Reach

L,L-cystathionine

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

739 compounds within 3 steps: 4 in 1, 155 in 2, 580 in 3

Does it reach a compound?

Two steps

hexadecanedioyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. Production of Hexadecanedioylcarnitine
Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes
pristanoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. fatty-acid-CoA ligase
Catalysed by NAGS, SLC27A2mitochondrial matrix, cytosolOne of 2 routes
undecanoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. medium-chain acyl-CoA ligase (undecylic acid)
Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 3 routes
thio-Mo-molybdopterinReach from here →
1. L-cystathionine cysteine-lyase (deaminating
2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
Catalysed by CTH, MOCOScytosol
(5Z)-tetradecenoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. long-chain-fatty-acid-CoA ligase (physeteric acid)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes
(3E)-decenoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial
Catalysed by NAGS, ACADM, ECHS1, ACAA2 and 1 moremitochondrial matrix
(2R,6)-dimethylheptanoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)
Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrix
(7Z)-hexadecenoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes
tricosanoyl-CoAReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. long-chain-fatty-acid-CoA ligase (tricosanoic acid)
Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

Three steps

beta-carbolineReach from here →
Catalysed by CTH, SLC7A5cytosol, extracellular space
N(omega)-methyl-L-arginineReach from here →
Catalysed by CTH, and 1 more

Page 4 of 15

SLC3A2

, SLC7A5

, SLC7A8

cytosol, extracellular space

One of 4 routes

3-methyl-2-oxobutanoateReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and valine (cytosol to extracellular)
L-Valine:2-oxoglutarate aminotransferase

Catalysed by CTH, SLC7A5, BCAT2cytosol, extracellular space, mitochondrial matrixOne of 3 routes

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

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Carnitine O-Palmitoyltransferase
carnitine O-palmitoyltransferase ((9E)-tetradecenoyl-CoA)

Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 7 routes

S-butanoyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Butanoate:CoA ligase (AMP-forming)
butanoyl-CoA + H2O = S-butanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 6 moremitochondrial matrix, cytosol

S-octanoyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Octanoyl-CoA:L-carnitine O-octanoyltransferase
octanoyl-CoA + H2O = S-octanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, CROT, NUDT19, NUDT7 and 1 moremitochondrial matrix

decanoyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
medium-chain acyl-CoA ligase (decanoic acid)
decanoyl-CoA + H2O = decanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

S-dodecanoyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
long-chain-fatty-acid-CoA ligase (lauric acid)
dodecanoyl-CoA + H2O = S-dodecanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

tetradecanoyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
long-chain-fatty-acid-CoA ligase (myristic acid)
tetradecanoyl-CoA + H2O = tetradecanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

an acyl-4'-phosphopantetheineReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
acyl-CoA:sphingosine N-acyltransferase
an acyl-CoA + H2O = an acyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus

tauroursodeoxycholateReach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
transport of GSH and tauroursodeoxycholate (cytosol to extracellular)

Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

glycoursodeoxycholateReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and glycine (cytosol to extracellular)
glycine to glycoursodeoxycholate conversion

Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular spaceOne of 4 routes

(11S,15S)-dihydroxy-14(R)-S-glutathionyl-(5Z,8Z,12E)-eicosatrienoateReach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
glutathione transferase (GSH)

Catalysed by CTH, PTGES, MGST3, GSTA4 and 18 morecytosolOne of 4 routes

prostaglandin E3Reach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
transport of GSH and prostaglandin E3 (cytosol to extracellular)

Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

prostaglandin A2Reach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
transport of GSH and prostaglandin A2 (cytosol to extracellular)

Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

prostaglandin B1Reach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
transport of GSH and prostaglandin B1 (cytosol to extracellular)

Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

leukotriene F4Reach from here →

L-cystathionine cysteine-lyase (deaminating
cystine to cysteine conversion
transport of GSH and leukotriene F4 (cytosol to extracellular)

Catalysed by CTHcytosol, extracellular spaceOne of 3 routes

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

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
N-acetyl-LTE4 to leukotriene E4 conversion
transport of leukotriene E4 (cytosol to extracellular)

Catalysed by NAGS, ZDHHC11B, NAA10, ESCO2 and 30 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

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

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and glycine (cytosol to extracellular)
(9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 2 morecytosol, extracellular space

N-(9Z-octadecenoyl)-L-phenylalanineReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and phenylalanine (cytosol to extracellular)
L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

Catalysed by CTH, SLC7A5, PM20D1cytosol, extracellular space

N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanineReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and phenylalanine (cytosol to extracellular)
(5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

Catalysed by CTH, SLC7A5, PM20D1cytosol, extracellular space

N-(9Z-octadecenoyl)-L-leucineReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and leucine (cytosol to extracellular)
N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

Catalysed by CTH, SLC7A9, SLC3A1, PM20D1cytosol, extracellular space

N-octadecanoylglycineReach from here →

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and glycine (cytosol to extracellular)
octadecanoyl-CoA + glycine = N-octadecanoylglycine + CoA + H(+)

Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

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

L-cystathionine cysteine-lyase (deaminating
transport of cysteine and glycine (cytosol to extracellular)
(9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

acetoacetateReach from here →

L-cystathionine cysteine-lyase (deaminating
2-hydroxybutyrate:NAD+ oxidoreductase
transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)

Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 6 routes

2-hydroxyoctanoyl-CoAReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Octanoyl-CoA:L-carnitine O-octanoyltransferase
octanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyoctanoyl-CoA + succinate + CO2

Catalysed by NAGS, CROT, PHYHmitochondrial matrix

2-hydroxydecanoyl-CoAReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
medium-chain acyl-CoA ligase (decanoic acid)
decanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxydecanoyl-CoA + succinate + CO2

Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

2-hydroxyeicosanoyl-CoAReach from here →

N-acetylglutamate synthase (N-acetyl-L-cystathionine)
long-chain-fatty-acid-CoA ligase (eicosanoate)
eicosanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyeicosanoyl-CoA + succinate + CO2