homocysteine

Three steps

• CHEBI:18095Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by MTR, SLC7A5, SLC1A4cytosol, extracellular spaceOne of 5 routes

• CHEBI:18183Reach from here →
  1. Formation of Homocystine
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by GGT1, GGT5, GGTLC2, GGT6 and 4 morecytosol, extracellular space

• N-carbamoyl-beta-alanineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of chloride and methionine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by MTR, SLC6A14cytosol, extracellular spaceOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by MTR, MTHFD1, GARTcytosol

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by MTR, SLC7A5, GARTcytosol, extracellular space

• CHEBI:18381Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by MTR, MTHFD1, ATICcytosolOne of 2 routes

• 2-phenylethylamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by MTR, SLC7A5, DDCcytosol, extracellular space

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by MTR, SLC7A5, PFAScytosol, extracellular space

• (6S)-5-methyl-5,6,7,8-tetrahydrofolateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)
  3. 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(in) + (6S)-5-methyl-5,6,7,8-tetrahydrofolate(out) = 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(out) + (6S)-5-methyl-5,6,7,8-tetrahydrofolate(in)

  Catalysed by MTR, ATIC, SLC19A1cytosol, inner side of the membrane, outer side of the membrane

• (R)-normetanephrineReach from here →
  1. Formation of Homocystine
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:catechol O-methyltransferase

  Catalysed by SLCO1A2, SLCO1B3, COMT, LRTOMTcytosol, extracellular space

• ophthalmateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by MTR, SLC7A5, GSScytosol, extracellular space

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by CBS, CTH, BCKDHA, BCKDHBcytosol, mitochondrial matrix

• 2-aminoisobutanoateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by MTR, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3',5'-diiodo-L-thyronineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2

  Catalysed by MTR, CDKAL1, DIO3cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate

  Catalysed by CBS, CTH, AGXT2cytosol

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by MTR, SLC7A5, AGXT2cytosol, extracellular spaceOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by CBS, CTH, CARS2cytosol

• (5Z)-dodecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by CBS, NAGS, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronate + UDP

  Catalysed by UGT1A9, UGT2B7, UGT1A6, UGT1A1 and 2 morecytosol, extracellular space

• 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2
  3. 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP + H(+)

  Catalysed by SLCO2A1, UGT2B7, UGT1A1, UGT1A4cytosol, extracellular space

• 15-O-(beta-D-glucuronosyl)-prostaglandin B1Reach from here →
  1. Formation of Homocystine
  2. transport of GSH and prostaglandin B1 (cytosol to extracellular)
  3. prostaglandin B1 + UDP-alpha-D-glucuronate = 15-O-(beta-D-glucuronosyl)-prostaglandin B1 + UDP + H(+)

  Catalysed by SLCO2A1, UGT1A9, UGT2B7, UGT1A1 and 1 morecytosol, extracellular space

• 3-iodothyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO3cytosolOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• 3-iodo-L-thyronineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO3cytosolOne of 2 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• 3'-iodo-L-thyronineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO2cytosolOne of 2 routes

• serotonin O-sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. serotonin + 3'-phosphoadenylyl sulfate = serotonin O-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4cytosol, extracellular space

• (R)-noradrenaline 4'-O-sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. sulfotransferase (noradrenaline)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3cytosol, extracellular spaceOne of 2 routes

• L-thyronineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by MTR, CDKAL1, DIO3cytosol

• thyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO3cytosol

• 3'-iodothyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO2cytosolOne of 2 routes

• 3,3'-diiodothyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO2cytosolOne of 2 routes

• 3',5'-diiodothyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO3cytosol

• 3,3',5'-triiodothyronamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 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(+)
  3. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by MTR, CDKAL1, DIO3cytosol

• L-homocysteinyl-AMPReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-homocysteine + L-serine = L,L-cystathionine + H2O
  3. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by CBS, MARS1cytosol

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by CBS, NAGS, HADHA, ACADVL and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by CBS, NAGS, HADHA, ACADVL and 1 morecytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by CBS, NAGS, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by CBS, NAGS, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• ursodeoxycholate 3-sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and ursodeoxycholate (cytosol to extracellular)
  3. ursodeoxycholate + 3'-phosphoadenylyl sulfate = ursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT2A1cytosol, extracellular space

• tauroursodeoxycholate 3-sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and tauroursodeoxycholate (cytosol to extracellular)
  3. tauroursodeoxycholate + 3'-phosphoadenylyl sulfate = tauroursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT2A1cytosol, extracellular space

• glycoursodeoxycholate 3-sulfateReach from here →
  1. Formation of Homocystine
  2. transport of glycoursodeoxycholate and GSH (cytosol to extracellular)
  3. glycoursodeoxycholate + 3'-phosphoadenylyl sulfate = glycoursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT2A1cytosol, extracellular space

• deoxycholateReach from here →
  1. Formation of Homocystine
  2. solute carrier organic anion transporter (cholate)
  3. cytochrome P450 (cholate)

  Catalysed by SLCO1A2, SLCO1B3, CYP7A1cytosol, extracellular spaceOne of 4 routes

• CHEBI:240943Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by MTR, SLC7A5, GLYAT, GLYATL2 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 3 routes

• hypochlorous acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of chloride and methionine (cytosol to extracellular)
  3. Peroxidase

  Catalysed by MTR, SLC6A14, LPOcytosol, extracellular spaceOne of 4 routes

• potassium atomReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of chloride and methionine (cytosol to extracellular)
  3. transport of chloride (cytosol to extracellular)

  Catalysed by MTR, SLC6A14, SLC12A2, SLC12A1cytosol, extracellular spaceOne of 4 routes

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamineReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. fatty acylamide amidohydrolase

  Catalysed by FAAHcytosol, extracellular space

• imidazole-4-acetaldehydeReach from here →
  1. Formation of Homocystine
  2. transport of GSH and histamine (cytosol to extracellular)
  3. amine oxidase copper containing (histamine)

  Catalysed by SLCO1A2, SLCO1B3, AOC2, AOC1 and 1 morecytosol, extracellular spaceOne of 2 routes

• folic acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase
  3. dihydrofolate:NAD+ oxidoreductase

  Catalysed by MTR, DHFR, DHFR2cytosolOne of 2 routes

• phenylacetylglycineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by MTR, SLC7A5, GLYATcytosol, extracellular spaceOne of 3 routes