glycine betaine

Three steps

• thioredoxin dithiolReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-Methionine:tRNAMet ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by BHMT, BHMT2, MARS1cytosolOne of 2 routes

• methanethiolReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

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

• ureaReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by BHMT, BHMT2, SLC7A6, ARG1cytosol, extracellular spaceOne of 3 routes

• succinic semialdehydeReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)
  3. 4-aminobutanoate:2-oxoglutarate aminotransferase

  Catalysed by SLC6A12, ABATextracellular space, cytosol, mitochondrial matrix

• L-cystineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A9 and 1 morecytosol, extracellular spaceOne of 9 routes

• 11-cis-retinolReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by BHMT, BHMT2, NAGS, DGAT1 and 4 morecytosol, mitochondrial matrix

• cob(II)alaminReach 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 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, MTRRcytosol

• (R)-3-aminoisobutyric acidReach 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 matrix

• oxaloacetateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. ATP citrate lyase (citrate)

  Catalysed by BHMT, BHMT2, NAGS, ACLYcytosol, mitochondrial matrixOne of 6 routes

• nitric oxideReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by BHMT, BHMT2, SLC7A6, NOS3 and 2 morecytosol, extracellular spaceOne of 3 routes

• CHEBI:16523Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by BHMT, BHMT2, SLC7A5, SLC3A2 and 1 morecytosol, extracellular spaceOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by BHMT, BHMT2, SLC7A6, ACY1cytosol, extracellular space

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

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

• CHEBI:16633Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

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

• quinolinic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of Quinolinic Acid

  Catalysed by SLC6A12, SLC26A6, SLC22A6extracellular space, cytosolOne of 2 routes

• 3'-deoxy-3',4'-didehydro-CTPReach 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. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by BHMT, BHMT2, CDKAL1, RSAD2cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by BHMT, BHMT2, SLC7A5, CNDP2cytosol, extracellular space

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

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

• tryptamineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

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

• 2-oxoglutaramateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A5, KYAT1cytosol, extracellular spaceOne of 3 routes

• (S)-2-hydroxyglutarateReach 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. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, L2HGDHcytosol

• CHEBI:16856Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by BHMT, BHMT2, SLC7A5, GSScytosol, extracellular spaceOne of 2 routes

• glyoxylic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by BHMT, BHMT2, SLC7A5, DAOcytosol, extracellular space, peroxisome

• creatineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Transport of Guanidinoacetic Acid
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC6A12, GAMTextracellular space, cytosolOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by BHMT, BHMT2, SLC7A13, NAT8Lcytosol, inner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of (R)-3-hydroxybutanoate and formate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC26A4, SLC16A4, SLC16A5 and 2 moreextracellular space, cytosolOne of 2 routes

• taurolithocholateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. bile acid-CoA:amino acid N-acyltransferase (lithocholate)

  Catalysed by SLC6A12, SLC6A6, BAATextracellular space, cytosolOne of 2 routes

• L-glutamic 5-semialdehydeReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A6, OATcytosol, extracellular space, mitochondrial matrix

• itaconateReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate

  Catalysed by SLC22A5, SLC22A8, SUGCTinner side of the membrane, outer side of the membrane

• anilineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by BHMT, BHMT2, NAGS, NAT2 and 1 morecytosol, mitochondrial matrix

• CHEBI:17312Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by BHMT, BHMT2, NAGScytosol, mitochondrial matrixOne of 2 routes

• 5'-deoxyadenosineReach 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. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)

  Catalysed by BHMT, BHMT2, CDKAL1, CDK5RAP1cytosolOne of 4 routes

• 3-hydroxy-3-methylglutarateReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by SLC22A5, SLC22A8, SUGCTinner side of the membrane, outer side of the membrane

• linoleic 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

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

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• D-aspartic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC12A2, SLC12A1, SLC1A7 and 4 moreextracellular space, cytosol

• CHEBI:17380Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by BHMT, BHMT2, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by BHMT, BHMT2, SLC7A5, PRODH2cytosol, extracellular spaceOne of 2 routes

• agmatineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by BHMT, BHMT2, SLC7A6cytosol, extracellular space, mitochondrial matrix

• 5-aminolevulinic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by BHMT, BHMT2, SLC7A5, ALAS1 and 2 morecytosol, extracellular space, mitochondrial matrix

• diiodineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and iodide (cytosol to extracellular)
  3. iodide:hydrogen-peroxide oxidoreductase

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

• indole-3-pyruvateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by BHMT, BHMT2, SLC7A5, IL4I1cytosol, extracellular spaceOne of 2 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach 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. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO1cytosol

• 3,3'-diiodo-L-thyronineReach 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. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO1 and 1 morecytosolOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach 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. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO1cytosolOne of 4 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by BHMT, BHMT2, NAGS, HADHA and 2 morecytosol, mitochondrial matrix

• Ala-tRNA(Ala)Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, SLC7A5, AARS1cytosol, extracellular space

• trans-urocanateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of histidine and methionine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

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

• urateReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. glutarate(in) + (R)-carnitine(out) = glutarate(out) + (R)-carnitine(in)
  3. urate(in) + glutarate(out) = urate(out) + glutarate(in)

  Catalysed by SLC22A5, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-hydroxy-L-tryptophanReach 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 space