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Human Metabolome Database Version 3.5

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Showing metabocard for Betaine (HMDB00043)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:48 -0700
HMDB ID HMDB00043
Secondary Accession Numbers None
Metabolite Identification
Common Name Betaine
Description Betaine or trimethylglycine is a methylated derivative of glycine. It functions as a methyl donor in that it carries and donates methyl functional groups to facilitate necessary chemical processes. The donation of methyl groups is important to proper liver function, cellular replication, and detoxification reactions. Betaine also plays a role in the manufacture of carnitine and serves to protect the kidneys from damage. Betaine has also been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th Ed, p1341).
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (Carboxymethyl)trimethylammonium hydroxide inner salt
  2. (Trimethylammonio)acetate
  3. 1-Carboxy-N,N,N-trimethyl-Methanaminium
  4. 1-Carboxy-N,N,N-trimethyl-Methanaminium hydroxide
  5. 1-Carboxy-N,N,N-trimethylmethanaminium inner salt
  6. a-Earleine
  7. Abromine
  8. alpha-Earleine
  9. Aminocoat
  10. Betafin
  11. Betafin BCR
  12. Betafin BP
  13. Betaine
  14. Cystadane
  15. Ektasolve EE
  16. FinnStim
  17. Glycine betaine
  18. Glycocoll betaine
  19. Glycylbetaine
  20. Greenstim
  21. Loramine AMB 13
  22. Loramine AMB-13
  23. Lycine
  24. N,N,N-Trimethylglycine
  25. Oxyneurine
  26. Rubrine C
  27. Trimethylaminoacetate
  28. Trimethylaminoacetic acid
  29. Trimethylbetaine Glycine
  30. Trimethylglycine
  31. Trimethylglycocoll
Chemical Formula C5H11NO2
Average Molecular Weight 117.1463
Monoisotopic Molecular Weight 117.078978601
IUPAC Name 2-(trimethylazaniumyl)acetate
Traditional IUPAC Name betaine
CAS Registry Number 107-43-7
SMILES C[N+](C)(C)CC([O-])=O
InChI Identifier InChI=1S/C5H11NO2/c1-6(2,3)4-5(7)8/h4H2,1-3H3
InChI Key KWIUHFFTVRNATP-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Amino Acids, Peptides, and Analogues
Class Amino Acids and Derivatives
Sub Class Alpha Amino Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • a methylated methyl acceptor(Cyc)
  • amino-acid betaine(ChEBI)
  • glycine derivative(ChEBI)
Substituents
  • Carboxylic Acid Salt
  • Quaternary Ammonium Salt
Direct Parent Alpha Amino Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Glycine, serine and threonine metabolism
  • Component of Methionine metabolism
  • Osmolyte
Application Not Available
Cellular locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 293 - 301 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 611.0 mg/mL at 19 °C Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 1.86 g/L ALOGPS
LogP -2.70 ALOGPS
LogP -4.5 ChemAxon
LogS -1.96 ALOGPS
pKa (strongest acidic) 2.26 ChemAxon
Hydrogen Acceptor Count 2 ChemAxon
Hydrogen Donor Count 0 ChemAxon
Polar Surface Area 40.13 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 52.82 ChemAxon
Polarizability 12.11 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
1H NMR Spectrum
13C NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50
MS/MS Spectrum LC-ESI-QTOF (UPLC Q-Tof Premier, Waters)
MS/MS Spectrum LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30
[1H,1H] 2D NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Skeletal Muscle
  • Bladder
  • Fibroblasts
  • Intestine
  • Pancreas
  • Placenta
  • Testes
  • Kidney
  • Liver
  • Prostate
  • Adrenal Gland
  • Skin
  • Adipose Tissue
  • Adrenal Cortex
  • Platelet
  • Gonads
  • Gut
  • Spleen
  • Stratum Corneum
Pathways
Name SMPDB Link KEGG Link
Glycine and Serine Metabolism SMP00004 map00260 Link_out
Betaine Metabolism SMP00123 map00260 Link_out
Methionine Metabolism SMP00033 map00270 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
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33.6 (23.9-42.1) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
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82.0 (20.0-144.0) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
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72 +/- 22.4 uM Adult (>18 years old) Not Specified Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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18.6 (4.868-32.4) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
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11.5 (2.7-24.7) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR
Urine Detected and Quantified
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4.14 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Male Normal After green tea sonsumption
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Male Normal After black tea sonsumption
Urine Detected and Quantified
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6.1 +/- 3.2 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
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49.6 (6.4-92.7) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
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42.1 +/- 19.3 uM Adult (>18 years old) Not Specified Heart Transplant Not Available
Blood Detected and Quantified
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32.5 (23.9-42.5) uM Adult (>18 years old) Both Chronic renal failure Not Available
Blood Detected and Quantified
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25.6 (23.4-36.6) uM Adult (>18 years old) Both Continuous ambulatory peritoneal dialysis Not Available
Blood Detected and Quantified
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31.9 (28.3-36.2) uM Adult (>18 years old) Both Hemodialysis Not Available
Urine Detected and Quantified
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6.0 (0.0-16.0) umol/mmol creatinine Adult (>18 years old) Both Lung cancer Not Available
Urine Detected and Quantified
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51.2 +/- 7.6 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency Not Available
Urine Detected and Quantified
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76.2 +/- 8 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency Not Available
Urine Detected and Quantified
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210.1 +/- 16.4 umol/mmol creatinine Adult (>18 years old) Both Arginosuccinic aciduria Not Available
Urine Detected and Quantified
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20.1 +/- 4.7 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia Not Available
Urine Detected and Quantified
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122 +/- 13.6 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia Not Available
Urine Detected and Quantified
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8.5 +/- 0.4 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria Not Available
Urine Detected and Quantified
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14.3 +/- 2.7 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria Not Available
Urine Detected and Quantified
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14.2 +/- 0.9 umol/mmol creatinine Adult (>18 years old) Both Maple syrup urine disease Not Available
Urine Detected and Quantified
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58 +/- 2.2 umol/mmol creatinine Adult (>18 years old) Both Aminoaciduria Not Available
Associated Disorders and Diseases
Disease References
Lung Cancer
    • HMP experimental
    Chronic renal failure
    • McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. Pubmed: 11380830 Link_out
      Continuous ambulatory peritoneal dialysis
      • McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. Pubmed: 11380830 Link_out
        Hemodialysis
        • McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. Pubmed: 11380830 Link_out
          Associated OMIM IDs
          DrugBank ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB009020
          KNApSAcK ID C00007291 Link_out
          Chemspider ID 242 Link_out
          KEGG Compound ID C00719 Link_out
          BioCyc ID BETAINE Link_out
          BiGG ID 35786 Link_out
          Wikipedia Link Betaine Link_out
          NuGOwiki Link HMDB00043 Link_out
          Metagene Link HMDB00043 Link_out
          METLIN ID 287 Link_out
          PubChem Compound 247 Link_out
          PDB ID Not Available
          ChEBI ID 17750 Link_out
          References
          Synthesis Reference Mu, Yun; Guo, Xiao-hui. Improved process for preparation of betaine. Huaxue Yu Shengwu Gongcheng (2005), 22(7), 48-49.
          Material Safety Data Sheet (MSDS) Download (PDF)
          General References
          1. Surtees R, Bowron A, Leonard J: Cerebrospinal fluid and plasma total homocysteine and related metabolites in children with cystathionine beta-synthase deficiency: the effect of treatment. Pediatr Res. 1997 Nov;42(5):577-82. Pubmed: 9357926 Link_out
          2. Holm PI, Ueland PM, Kvalheim G, Lien EA: Determination of choline, betaine, and dimethylglycine in plasma by a high-throughput method based on normal-phase chromatography-tandem mass spectrometry. Clin Chem. 2003 Feb;49(2):286-94. Pubmed: 12560353 Link_out
          3. Knopman D, Patterson M: An open-label, 24-week pilot study of the methyl donor betaine in Alzheimer disease patients. Alzheimer Dis Assoc Disord. 2001 Jul-Sep;15(3):162-5. Pubmed: 11522934 Link_out
          4. Schwahn BC, Chen Z, Laryea MD, Wendel U, Lussier-Cacan S, Genest J Jr, Mar MH, Zeisel SH, Castro C, Garrow T, Rozen R: Homocysteine-betaine interactions in a murine model of 5,10-methylenetetrahydrofolate reductase deficiency. FASEB J. 2003 Mar;17(3):512-4. Epub 2003 Jan 22. Pubmed: 12551843 Link_out
          5. Abdelmalek MF, Angulo P, Jorgensen RA, Sylvestre PB, Lindor KD: Betaine, a promising new agent for patients with nonalcoholic steatohepatitis: results of a pilot study. Am J Gastroenterol. 2001 Sep;96(9):2711-7. Pubmed: 11569700 Link_out
          6. Abu Al-Soud W, Radstrom P: Effects of amplification facilitators on diagnostic PCR in the presence of blood, feces, and meat. J Clin Microbiol. 2000 Dec;38(12):4463-70. Pubmed: 11101581 Link_out
          7. Sakamoto A, Ono H, Mizoguchi N, Sakura N: Betaine and homocysteine concentrations in infant formulae and breast milk. Pediatr Int. 2001 Dec;43(6):637-40. Pubmed: 11737741 Link_out
          8. Schwab U, Torronen A, Toppinen L, Alfthan G, Saarinen M, Aro A, Uusitupa M: Betaine supplementation decreases plasma homocysteine concentrations but does not affect body weight, body composition, or resting energy expenditure in human subjects. Am J Clin Nutr. 2002 Nov;76(5):961-7. Pubmed: 12399266 Link_out
          9. Chambers ST, Kunin CM: Isolation of glycine betaine and proline betaine from human urine. Assessment of their role as osmoprotective agents for bacteria and the kidney. J Clin Invest. 1987 Mar;79(3):731-7. Pubmed: 3546377 Link_out
          10. Laryea MD, Steinhagen F, Pawliczek S, Wendel U: Simple method for the routine determination of betaine and N,N-dimethylglycine in blood and urine. Clin Chem. 1998 Sep;44(9):1937-41. Pubmed: 9732980 Link_out
          11. Chung YL, Rider LG, Bell JD, Summers RM, Zemel LS, Rennebohm RM, Passo MH, Hicks J, Miller FW, Scott DL: Muscle metabolites, detected in urine by proton spectroscopy, correlate with disease damage in juvenile idiopathic inflammatory myopathies. Arthritis Rheum. 2005 Aug 15;53(4):565-70. Pubmed: 16082628 Link_out
          12. Go EK, Jung KJ, Kim JY, Yu BP, Chung HY: Betaine suppresses proinflammatory signaling during aging: the involvement of nuclear factor-kappaB via nuclear factor-inducing kinase/IkappaB kinase and mitogen-activated protein kinases. J Gerontol A Biol Sci Med Sci. 2005 Oct;60(10):1252-64. Pubmed: 16282556 Link_out
          13. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 Link_out
          Enzymes
          Name: Choline dehydrogenase, mitochondrial
          Reactions:
          • choline + acceptor = betaine aldehyde + reduced acceptor [RN:R01025]
          Gene Name: CHDH
          Uniprot ID: Q8NE62 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Betaine--homocysteine S-methyltransferase 1
          Reactions:
          • trimethylammonioacetate + L-homocysteine = dimethylglycine + L-methionine [RN:R02821]
          Gene Name: BHMT
          Uniprot ID: Q93088 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Sodium- and chloride-dependent betaine transporter
          Reactions:
            Gene Name: SLC6A12
            Uniprot ID: P48065 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA
            Name: Betaine--homocysteine S-methyltransferase 2
            Reactions:
            • trimethylammonioacetate + L-homocysteine = dimethylglycine + L-methionine [RN:R02821]
            Gene Name: BHMT2
            Uniprot ID: Q9H2M3 Link_out
            Protein Sequence: FASTA
            Gene Sequence: FASTA