glycine betaine

Three steps

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

  Catalysed by BHMT, BHMT2, SLC3A2, SLC7A5 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

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

• 3-oxopropanoic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of beta-alanine and chloride (cytosol to extracellular)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC6A12, SLC6A6, SLC6A14, ABATextracellular space, cytosol, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by BHMT, BHMT2, SLC7A5, GOT2 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 5 routes

• fumaric acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of fumarate and sulfate (cytosol to mitochondria)

  Catalysed by SLC6A12, SLC26A6, SLC25A10extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:18132Reach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. ATP:choline phosphotransferase

  Catalysed by SLC6A12, SLC5A7, CHKA, CHKBextracellular space, cytosol

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

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

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

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

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

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

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

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

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach 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. 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(out) + glutarate(in) = 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(in) + glutarate(out)

  Catalysed by SLC22A5, SLC22A8, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

  Catalysed by BHMT, BHMT2, CDKAL1, DIO3cytosol

• 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 2 routes

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. formaldehyde to 5,10-methylene-THF conversion

  Catalysed by BHMT, BHMT2, PDPR, DMGDHcytosolOne of 2 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. L-Methionine:tRNAMet ligase (AMP-forming)
  3. 10-Formyltetrahydrofolate:L-methionyl-tRNA N-formyltransferase

  Catalysed by BHMT, BHMT2, MARS1, MTFMTcytosolOne of 3 routes

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

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

• (5Z)-dodecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

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

• 3-iodothyronamineReach 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-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

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

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 5 routes

• 3-iodo-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-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

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

• (7Z)-tetradecenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 8 routes

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

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

• 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. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO3cytosol

• thyronamineReach 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. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO3cytosol

• 3'-iodothyronamineReach 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'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

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

• 3,3'-diiodothyronamineReach 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'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

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

• 3',5'-diiodothyronamineReach 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',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO3cytosol

• 3,3',5'-triiodothyronamineReach 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'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by BHMT, BHMT2, CDKAL1, DIO3cytosol

• L-homocysteine thiolactoneReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP = L-homocysteine thiolactone + AMP + diphosphate

  Catalysed by BHMT, BHMT2, MARS1cytosol

• L-homocysteinyl-AMPReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by BHMT, BHMT2, MARS1cytosol

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

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

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

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

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by BHMT, BHMT2, NAGS, ECHS1 and 2 morecytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by BHMT, BHMT2, NAGS, ECHS1 and 2 morecytosol, mitochondrial matrixOne of 2 routes

• alpha-linolenic 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 reticulum

• folic acidReach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Folate Transport by Oats

  Catalysed by SLC6A12, SLC26A6, SLC22A8, SLC22A7extracellular space, cytosolOne of 2 routes

• phenylacetylglycineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by BHMT, BHMT2, NAGS, ACSM4 and 5 morecytosol, mitochondrial matrixOne of 6 routes

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

  Catalysed by BHMT, BHMT2, NAGS, SLC27A2 and 7 morecytosol, mitochondrial matrixOne of 5 routes

• 5-hydroxytryptophanReach 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 2 routes

• CHEBI:28666Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by BHMT, BHMT2, SLC7A5cytosol, extracellular space

• CHEBI:28701Reach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by BHMT, BHMT2, NAGS, ACAA1 and 2 morecytosol, mitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach 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

• N-acetyl-L-cysteineReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

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

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

  Catalysed by SLC6A12, SLC26A6, SLC25A35, SLC25A34extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by BHMT, BHMT2, SLC7A5, SLC1A1cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 4 routes

• Ca(2+)Reach from here →
  1. transport of betaine and chloride (cytosol to extracellular)
  2. transport of chloride (cytosol to extracellular)
  3. transport of Ca2+ and K+ (cytosol to extracellular)

  Catalysed by SLC6A12, SLC12A2, SLC12A1, SLC24A1 and 4 moreextracellular space, cytosol

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

  Catalysed by BHMT, BHMT2, SLC7A5, CARS1cytosol, extracellular space

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

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