glycine betaine

Three steps

• N-sulfo-D-glucosamineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC6A12, SLC26A6, SGSHextracellular space, cytosol

• dehydroepiandrosterone 3-sulfateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by SLC6A12, SLC26A6, STSextracellular space, cytosolOne of 3 routes

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

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

• glutathioneReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

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

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of lysine and methionine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

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

• L-kynurenineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

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

• leukotriene C4Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by BHMT, BHMT2, SLC7A5cytosol, extracellular space

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

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

• L-glutamate 5-semialdehydeReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

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

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

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

• L-cysteateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by SLC6A12, SLC6A6, GADL1, CSADextracellular space, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

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

• L,L-cystathionineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

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

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

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

• 6-amino-2-oxohexanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of lysine and methionine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by BHMT, BHMT2, SLC7A6cytosol, extracellular space, peroxisome

• carbamoyl phosphateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by BHMT, BHMT2, SLC7A5, CADcytosol, extracellular spaceOne of 4 routes

• adenosine 5'-phosphosulfateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. ATP:sulfate adenylyltransferase

  Catalysed by SLC6A12, SLC26A6, PAPSS1, PAPSS2extracellular space, cytosolOne of 4 routes

• L-tyrosineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by BHMT, BHMT2, SLC7A5, AADATcytosol, extracellular spaceOne of 4 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. Trimethylaminoacetate:L-homosysteine 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 BHMT, BHMT2, SLC7A5, AGXT2cytosol, extracellular spaceOne of 7 routes

• an acyl-CoAReach 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

• trans-4-hydroxy-L-prolineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by BHMT, BHMT2, SLC7A5, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by BHMT, BHMT2, SLC7A13, PAICScytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-thyroxineReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and iodide (cytosol to extracellular)
  3. 3,3',5-triiodo-L-thyronine,iodide:acceptor oxidoreductase (iodinating)

  Catalysed by SLC6A12, SLC26A4, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• kynurenateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O

  Catalysed by BHMT, BHMT2, AADATcytosolOne of 3 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by BHMT, BHMT2, SLC7A6, IL4I1cytosol, extracellular space

• (3Z)-dodecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

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

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

  Catalysed by BHMT, BHMT2, SLC7A5, CARS2cytosol, extracellular space

• N-formyl-L-kynurenineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

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

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 1 morecytosol, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

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

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

• 5-amino-2-oxopentanoateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by BHMT, BHMT2, SLC7A6, AGXT2cytosol, extracellular spaceOne of 2 routes

• deoxycholoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 1 morecytosol, mitochondrial matrixOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by BHMT, BHMT2, SLC7A5, GLYATL2cytosol, extracellular spaceOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by BHMT, BHMT2, SLC7A5, PPCScytosol, extracellular spaceOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

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

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by SLC6A12, SLC26A6, STSextracellular space, cytosolOne of 3 routes

• prostaglandin E2Reach 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 E2(out) + glutarate(in) = prostaglandin E2(in) + glutarate(out)

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

• N-benzoylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by BHMT, BHMT2, SLC7A5cytosol, extracellular space, mitochondrial matrixOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by BHMT, BHMT2, SLC7A5, CDO1cytosol, extracellular spaceOne of 6 routes

• decanoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by BHMT, BHMT2, NAGS, ACSM4 and 5 morecytosol, mitochondrial matrixOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 2 morecytosol, mitochondrial matrixOne of 4 routes

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

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

• L-cysteinylglycineReach 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, GGTLC3 and 5 morecytosol, extracellular spaceOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by BHMT, BHMT2, NAGS, CPT1A and 1 morecytosol, mitochondrial matrix

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by SLC6A12, CLTRN, SLC6A20, PYCR1 and 2 moreextracellular space, cytosolOne of 4 routes

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by SLC6A12, SLC26A6, SLC4A1extracellular space, cytosolOne of 2 routes