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

One step

CHEBI:1146900Reach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
Catalysed by NAGSmitochondrial matrix
2-oxobutanoateReach from here →
1. L-cystathionine cysteine-lyase (deaminating
Catalysed by CTHcytosolOne of 2 routes
L-cysteineReach from here →
1. L-cystathionine cysteine-lyase (deaminating
Catalysed by CTHcytosolOne of 2 routes
L-homocysteineReach from here →
1. L-homocysteine + L-serine = L,L-cystathionine + H2O
Catalysed by CBS

Two steps

(S)-carnitineReach from here →
1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
2. Carnitine O-Palmitoyltransferase
Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 244 routes
2-hydroxybutyric acidReach from here →
1. L-cystathionine cysteine-lyase (deaminating
2. 2-hydroxybutyrate:NAD+ oxidoreductase
Catalysed by CTH, LDHB, LDHC, LDHAL6A and 1 more

cytosol

AReach from here →

Catalysed by CTH, MOCOScytosol

(7Z)-octadecenoyl-CoAReach from here →

Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

CHEBI:15345Reach from here →

Catalysed by NAGS, AACSmitochondrial matrix, cytosolOne of 2 routes

cholineReach from here →

Catalysed by NAGS, CHATmitochondrial matrix, nucleus

pyruvateReach from here →

Catalysed by CTH, AGXT2cytosolOne of 3 routes

CHEBI:15380Reach from here →

Catalysed by NAGS, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

CHEBI:15477Reach from here →

Catalysed by NAGS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

2-methylcrotonoyl-CoAReach from here →

Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

isobutyryl-CoAReach from here →

Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

3-methylbut-2-enoyl-CoAReach from here →

Catalysed by NAGS, GLYATmitochondrial matrix

isovaleryl-CoAReach from here →

Catalysed by NAGS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

arachidonoyl-CoAReach from here →

Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

CHEBI:15519Reach from here →

Catalysed by NAGS, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

palmitoyl-CoAReach from here →

Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

oleoyl-CoAReach from here →

Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

phenylacetyl-CoAReach from here →

Catalysed by NAGSmitochondrial matrix, cytosolOne of 3 routes

propionyl-CoAReach from here →

Catalysed by CTHcytosolOne of 44 routes

L-leucineReach from here →

Catalysed by CTH, SLC7A9, SLC3A1cytosol, extracellular spaceOne of 3 routes

CHEBI:15650Reach from here →

Catalysed by NAGS, ZDHHC11B, NAA10, ESCO2 and 29 moremitochondrial matrix, cytosolOne of 2 routes

CHEBI:15699Reach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 3 routes

CHEBI:15727Reach from here →

Catalysed by NAGS, NAA10mitochondrial matrix, cytosol

CHEBI:15746Reach from here →

Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

CHEBI:15971Reach from here →

Catalysed by CTH, SLC7A6cytosol, extracellular spaceOne of 2 routes

L-cystineReach from here →

Catalysed by CTH, TXNRD1, TXNRD3, TXNRD2cytosolOne of 4 routes

11-cis-retinolReach from here →

Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

L-citrullineReach from here →

Catalysed by CTH, SLC7A6cytosol, extracellular spaceOne of 2 routes

L-valineReach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 3 routes

oxaloacetateReach from here →

Catalysed by NAGS, ACLYmitochondrial matrix, cytosol

L-arginineReach from here →

Catalysed by CTH, SLC7A6cytosol, extracellular spaceOne of 5 routes

protein polypeptide chainReach from here →

Catalysed by CTHcytosol

CHEBI:16856Reach from here →

Catalysed by CTHcytosol

CHEBI:16857Reach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 4 routes

L-isoleucineReach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 4 routes

CHEBI:17203Reach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 3 routes

anilineReach from here →

Catalysed by NAGS, NAT2, NAT1mitochondrial matrix, cytosol

CHEBI:17312Reach from here →

Catalysed by NAGSmitochondrial matrix, cytosolOne of 2 routes

(7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →

Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrix

CHEBI:17895Reach from here →

Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 4 routes

CHEBI:17927Reach from here →

Catalysed by NAGSmitochondrial matrix, cytosol

CHEBI:18019Reach from here →

Catalysed by CTH, SLC7A6cytosol, extracellular spaceOne of 2 routes

L-glutamineReach from here →

Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10cytosol, extracellular spaceOne of 4 routes

2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →

Catalysed by CTH, BCKDHA, BCKDHBcytosol, mitochondrial matrix

5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →

Catalysed by CTH, AGXT2cytosol

S-disulfanyl-L-cysteineReach from here →

Catalysed by CTH, CARS2cytosol