glycine betaine

Three steps

• 2-(prostaglandin H2)-sn-glycero-3-phosphocholineReach from here →
  1. Trimethylaminoacetate:L-homosysteine 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. 2-(prostaglandin G2)-sn-glycero-3-phosphocholine + AH2 = 2-(prostaglandin H2)-sn-glycero-3-phosphocholine + A + H2O

  Catalysed by BHMT, BHMT2, CDKAL1, PTGS2cytosol

• an aryl sulfateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. an aryl sulfate + H2O = a phenol + sulfate + H(+)

  Catalysed by SLC6A12, SLC26A6, ARSL, ARSF and 4 moreextracellular space, cytosol

• N-hexadecanoylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL3cytosol, extracellular space

• N-dodecanoylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular space

• N-decanoylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular space

• N-octanoylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular space

• D-histidineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. D-histidine(in) + L-ornithine(out) + H(+)(in) = D-histidine(out) + L-ornithine(in) + H(+)(out)

  Catalysed by BHMT, BHMT2, SLC7A6, SLC25A2cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-homoarginineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-homoarginine(in) + L-arginine(out) = L-homoarginine(out) + L-arginine(in)

  Catalysed by BHMT, BHMT2, SLC7A6, SLC25A29cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of histidine and methionine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by BHMT, BHMT2, SLC7A6, CARNS1cytosol, extracellular spaceOne of 3 routes

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GARTcytosol, extracellular space

• L-cystine-bis-glycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by BHMT, BHMT2, SLC7A5, DPEP1 and 1 morecytosol, extracellular space

• glycyldehydrophenylalanineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by BHMT, BHMT2, SLC7A5, DPEP1cytosol, extracellular space

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, PFAScytosol, extracellular space

• kinetensinReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by BHMT, BHMT2, SLC7A5, CMA1cytosol, extracellular spaceOne of 3 routes

• an N-(fatty acyl)-glycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular space

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular space

• (7Z)-octadecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 2 morecytosol, mitochondrial matrixOne of 3 routes

• acetaldehydeReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by BHMT, BHMT2, SLC7A5cytosol, extracellular space

• CHEBI:15345Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, NAGS, AACScytosol, mitochondrial matrixOne of 2 routes

• acetylcholineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by SLC6A12, SLC5A7, CHATextracellular space, cytosolOne of 3 routes

• pyruvateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A5, AGXT2cytosol, extracellular space, mitochondrial matrixOne of 18 routes

• all-trans-retinoic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of formate and retinoate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC26A4, SLC16A4, SLC16A5extracellular space, cytosolOne of 2 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by BHMT, BHMT2, SLC7A5, TPH1 and 1 morecytosol, extracellular spaceOne of 3 routes

• CHEBI:15380Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by BHMT, BHMT2, NAGS, ALAS1 and 1 morecytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by BHMT, BHMT2, NAGS, GLYAT and 2 morecytosol, mitochondrial matrixOne of 3 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of Tiglylcarnitine

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 1 morecytosol, mitochondrial matrixOne of 2 routes

• isobutyryl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by BHMT, BHMT2, NAGS, HADHA and 2 morecytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by BHMT, BHMT2, NAGS, GLYATcytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by BHMT, BHMT2, NAGS, GLYAT and 2 morecytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 11 routes

• CHEBI:15519Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by BHMT, BHMT2, NAGS, ACAA1 and 2 morecytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 15 routes

• oleoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 2 morecytosol, mitochondrial matrixOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Phenylacetate:CoA ligase

  Catalysed by BHMT, BHMT2, NAGScytosol, mitochondrial matrixOne of 3 routes

• propionyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, NAGS, ACSS3 and 2 morecytosol, mitochondrial matrixOne of 45 routes

• CHEBI:15570Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by BHMT, BHMT2, SLC7A5, SLC3A2 and 1 morecytosol, extracellular spaceOne of 5 routes

• (S)-malateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of malate and sulfate (cytosol to mitochondria)

  Catalysed by SLC6A12, SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by SLC6A12, SLC26A4, MTHFD1extracellular space, cytosolOne of 2 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by BHMT, BHMT2, SLC7A5, TPH1 and 1 morecytosol, extracellular spaceOne of 2 routes

• CHEBI:15650Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by BHMT, BHMT2, NAGS, ZDHHC11B and 31 morecytosol, mitochondrial matrixOne of 2 routes

• betaine aldehydeReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. choline dehydrogenase (choline)

  Catalysed by SLC6A12, SLC5A7, CHDHextracellular space, cytosolOne of 3 routes

• trimethylenediamineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of lysine and methionine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by BHMT, BHMT2, SLC7A6, DHPScytosol, extracellular space

• CHEBI:15727Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by BHMT, BHMT2, NAGS, NAA10cytosol, mitochondrial matrixOne of 3 routes

• formateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. formaldehyde + NAD(+) + H2O = formate + NADH + 2 H(+)

  Catalysed by BHMT, BHMT2, PDPR, DMGDH and 1 morecytosol

• CHEBI:15746Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by BHMT, BHMT2, NAGS, SAT1 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• hexadecanoic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Carnitine Transport by Atb0
  3. Utilized Transport

  Catalysed by SLC6A12, SLC6A14extracellular space, cytosol, endoplasmic reticulumOne of 2 routes

• CHEBI:15765Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by BHMT, BHMT2, SLC7A5, THcytosol, extracellular spaceOne of 5 routes

• malonateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. malonate(in) + sulfate(out) = malonate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30 and 1 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by BHMT, BHMT2, SLC7A13, CADcytosol, inner side of the membrane, outer side of the membrane

• alpha-D-glucosamine 6-phosphateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by BHMT, BHMT2, SLC7A5, GFPT2 and 1 morecytosol, extracellular space