L-homocysteine
362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API
512 compounds within 3 steps: 8 in 1, 58 in 2, 446 in 3
Three steps
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (nervonic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (ximenic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (DHA)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (margaric acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (nonadecylic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Activation of dodecanedioic acid
Catalysed by CBS, NAGSmitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Production of Sebacoylcarnitine
Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Production of Suberylcarnitine
Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Activation of adipic acid for Formation of Adipoyl Carnitine
Catalysed by CBS, NAGSmitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Production of 3-Hydroxyoctadecenoylcarnitinec
Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal
Catalysed by CBS, NAGS, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal
Catalysed by CBS, NAGS, ACAA1, HSD17B4 and 2 moremitochondrial matrix, peroxisome
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Production of Hexadecanedioylcarnitine
Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- fatty-acid-CoA ligase
Catalysed by CBS, NAGS, SLC27A2mitochondrial matrix, cytosolOne of 2 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- medium-chain acyl-CoA ligase (undecylic acid)
Catalysed by CBS, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 3 routes
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- (9Z,12Z)-octadecadienoate + AH2 + O2 = (9S)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- (9Z,12Z)-octadecadienoate + AH2 + O2 = (9R)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + A + H2O
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O
- L-homocysteine + H2O = 2-oxobutanoate + hydrogen sulfide + NH4(+) + H(+)
- thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
- (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (physeteric acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial
Catalysed by CBS, NAGS, ACADM, ECHS1 and 2 moremitochondrial matrix
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- 2-glyceryl-prostaglandin G2 + AH2 = 2-glyceryl-prostaglandin H2 + A + H2O
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)
Catalysed by CBS, NAGS, HADHA, ACAA2 and 1 moremitochondrial matrix
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- transport of glutamine and methionine (cytosol to extracellular)
- an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)
Catalysed by MTR, SLC7A5, GLYATL1B, GLYATL1extracellular space, cytosol
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes
- L-homocysteine + L-serine = L,L-cystathionine + H2O
- N-acetylglutamate synthase (N-acetyl-L-cystathionine)
- long-chain-fatty-acid-CoA ligase (tricosanoic acid)
Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes
- L-homocysteine + H2O = 2-oxobutanoate + hydrogen sulfide + NH4(+) + H(+)
- thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
- (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + glutathione disulfide + H2O
- L-homocysteine + H2O = 2-oxobutanoate + hydrogen sulfide + NH4(+) + H(+)
- thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
- (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O
- L-homocysteine + H2O = 2-oxobutanoate + hydrogen sulfide + NH4(+) + H(+)
- thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
- RX + glutathione = an S-substituted glutathione + a halide anion + H(+)
Catalysed by CTH, TSTD1, GSTT4, GSTA4 and 21 moreOne of 2 routes
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O
- (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
- N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
- (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoate + A + H2O