glycine betaine

Three steps

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

  Catalysed by BHMT, BHMT2, SLC7A5, AADATcytosol, extracellular spaceOne of 12 routes

• glycolithocholic 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 (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by BHMT, BHMT2, SLC7A5, GCATcytosol, extracellular space, mitochondrial matrix

• (S)-lactic 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 L-lactate (cytosol to extracellular)

  Catalysed by SLC6A12, SLC26A4, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosolOne of 2 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by BHMT, BHMT2, CDKAL1, CH25Hcytosol

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by BHMT, BHMT2, AADAT, ADI1cytosol

• gamma-Glu-AlaReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by BHMT, BHMT2, SLC7A5, GGT1 and 5 morecytosol, extracellular space

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by BHMT, BHMT2, SLC7A5, GLDC and 1 morecytosol, extracellular space, mitochondrial matrix

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, RETSATcytosol

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

  Catalysed by BHMT, BHMT2, CDKAL1, DIO1 and 1 morecytosolOne of 5 routes

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

  Catalysed by BHMT, BHMT2, CDKAL1, DIO1 and 1 morecytosol

• (R)-methylmalonyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by BHMT, BHMT2, NAGS, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by BHMT, BHMT2, NAGScytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by BHMT, BHMT2, NAGS, ACP2cytosol, mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. (2E)-dodecenoyl-CoA formation

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

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by BHMT, BHMT2, NAGS, ACAA1 and 1 morecytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

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

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

  Catalysed by BHMT, BHMT2, NAGS, ACSM4 and 5 morecytosol, mitochondrial matrixOne of 11 routes

• dodecanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 13 routes

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

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 2 morecytosol, mitochondrial matrixOne of 6 routes

• hexadecanoyl-CoAReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA
  3. (R)-carnitine + hexadecanoyl-CoA = O-hexadecanoyl-(R)-carnitine + CoA

  Catalysed by SLC22A5, CPT2, CPT1A, CPT1Binner side of the membrane, outer side of the membrane

• eicosanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 13 routes

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

  Catalysed by BHMT, BHMT2, NAGS, SUCLG1 and 2 morecytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 12 routes

• octanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by BHMT, BHMT2, NAGS, CROTcytosol, mitochondrial matrixOne of 23 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)
  2. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA
  3. (R)-carnitine + (9Z)-octadecenoyl-CoA = O-(9Z)-octadecenoyl-(R)-carnitine + CoA

  Catalysed by SLC22A5, CPT2inner side of the membrane, outer side of the membrane

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

  Catalysed by BHMT, BHMT2, NAGScytosol, mitochondrial matrixOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 6 morecytosol, mitochondrial matrixOne of 16 routes

• prostaglandin F2alphaReach 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. prostaglandin F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)

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

• prostaglandin H2Reach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by BHMT, BHMT2, CDKAL1, PTGS1 and 1 morecytosolOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach 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. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by BHMT, BHMT2, CDKAL1, TBXAS1 and 2 morecytosol

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

• 7,8-dihydrofolateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by SLC6A12, SLC6A6, MTO1extracellular space, cytosol

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by BHMT, BHMT2, SLC7A5, SHMT1 and 1 morecytosol, extracellular spaceOne of 2 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by BHMT, BHMT2, SLC7A6, ASLcytosol, extracellular spaceOne of 6 routes

• carnosineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC6A12, SLC6A6, SLC6A14, CARNS1extracellular space, cytosol

• 4-guanidinobutanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by BHMT, BHMT2, SLC7A6, GATMcytosol, extracellular spaceOne of 2 routes

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by BHMT, BHMT2, SLC7A13, ADSS2 and 1 morecytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by BHMT, BHMT2, SLC7A6, GATMcytosol, extracellular spaceOne of 2 routes

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 7 routes

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

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

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL3 and 2 morecytosol, extracellular space

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by BHMT, BHMT2, SLC7A5, GOT2 and 1 morecytosol, extracellular spaceOne of 3 routes

• S-formylglutathioneReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by BHMT, BHMT2, PDPR, DMGDH and 6 morecytosol

• succinate 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 = succinate semialdehyde + L-glutamate

  Catalysed by SLC6A12, ABATextracellular space, cytosol

• sphing-4-enineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by BHMT, BHMT2, NAGS, ASAH1 and 3 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

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

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

  Catalysed by BHMT, BHMT2, NAGS, SAT1 and 1 morecytosol, mitochondrial matrixOne of 3 routes