glycine betaine

Three steps

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

  Catalysed by BHMT, BHMT2, SLC7A5, GARS1cytosol, extracellular space

• Asp-tRNA(Asp)Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, SLC7A13, DARS1cytosol, inner side of the membrane, outer side of the membrane

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

  Catalysed by BHMT, BHMT2, SLC7A5, WARS1cytosol, extracellular space

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

  Catalysed by BHMT, BHMT2, SLC7A5, IARS1cytosol, extracellular space

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

  Catalysed by BHMT, BHMT2, SLC7A5, QARS1cytosol, extracellular space

• isocyanateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by BHMT, BHMT2, SLC7A6, LACC1cytosol, extracellular space

• hypoioditeReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and iodide (cytosol to extracellular)
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by SLC6A12, SLC26A4, MPO, LPOextracellular space, cytosol

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

  Catalysed by BHMT, BHMT2, SLC7A5, NARS1cytosol, extracellular space

• hydrogenselenideReach 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. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

  Catalysed by BHMT, BHMT2, CDKAL1, SCLYcytosol

• phosphocholineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by SLC6A12, SLC5A7, PHOSPHO1extracellular space, cytosolOne of 2 routes

• glycocholateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by BHMT, BHMT2, SLC7A5, BAATcytosol, extracellular space, peroxisomeOne of 5 routes

• hydrogen sulfideReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

• D-aspartateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• acetateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of acetate and formate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC26A4, SLC16A4, SLC16A5 and 1 moreextracellular space, cytosolOne of 2 routes

• CHEBI:30249Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by BHMT, BHMT2, SLC7A5, IDO1 and 2 morecytosol, extracellular space

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

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC26A2 and 2 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• propionic 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 propanoate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC26A4, SLC16A4, SLC16A5 and 1 moreextracellular space, cytosolOne of 2 routes

• citric acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC6A12, SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• butyric acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of butyrate and formate (cytosol to extracellular)

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

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

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (9Z)-octadecenoateReach 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

• adipic acidReach 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. hexanedioate + glutaryl-CoA = hexanedioyl-CoA + glutarate

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

• isocitric acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC6A12, SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• (2R,3R)-tartrateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by SLC6A12, SLC26A6, SLC25A14, SLC25A30extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (9Z)-hexadecenoateReach 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 6 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach 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

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

  Catalysed by BHMT, BHMT2, SLC3A2, SLC7A5 and 1 morecytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 5 routes

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

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

• putrescineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by BHMT, BHMT2, SLC7A6, SLC3A2 and 1 morecytosol, extracellular spaceOne of 4 routes

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

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

• cis-aconitic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and oxalate (cytosol to extracellular)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by SLC6A12, SLC26A6, SLC25A1extracellular space, cytosol, mitochondrial matrixOne of 3 routes

• N-carbamoyl-L-aspartateReach 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 = N-carbamoyl-L-aspartate + phosphate + H(+)

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

• 3-oxopropanoateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by SLC6A12, SLC6A6, SLC6A14, AGXT2extracellular space, cytosol

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

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

• di-mu-sulfido-diiron(2+)Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by BHMT, BHMT2, SLC7A5, NDUFAB1 and 6 morecytosol, extracellular space, mitochondrial matrix

• serotoninReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of serotonin (cytosol to extracellular)

  Catalysed by SLC6A12, SLC12A2, SLC12A1, SLC6A4extracellular space, cytosol

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of isoleucine and methionine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by BHMT, BHMT2, SLC7A5, BCAT2 and 1 morecytosol, extracellular space

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 10 routes

• an acylcholineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by SLC6A12, SLC5A7, BCHEextracellular space, cytosol

• 5-aminolevulinateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by BHMT, BHMT2, SLC7A5, ALAS1 and 1 morecytosol, extracellular space

• 3-methyl-2-oxovaleric acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of isoleucine and methionine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A5, BCAT1cytosol, extracellular spaceOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A5, GOT2 and 2 morecytosol, extracellular spaceOne of 3 routes

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

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

• taurocholateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. Choloyl-CoA:glycine N-choloyltransferase

  Catalysed by SLC6A12, SLC6A6, BAATextracellular space, cytosol, peroxisomeOne of 5 routes

• taurodeoxycholateReach 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 (taurodeoxycholate)

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

• glycochenodeoxycholic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by BHMT, BHMT2, SLC7A5, BAATcytosol, extracellular space, peroxisomeOne of 3 routes