Human Metabolome Database Version 3.5

Showing metabocard for Betaine (HMDB00043)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-07-12 10:32:57 -0600
HMDB ID HMDB00043
Secondary Accession Numbers None
Metabolite Identification
Common Name Betaine
Description Betaine, (or N,N,N-trimethylglycine) was named after its discovery in sugar beet (Beta vulgaris) in the 19th century. It is a small N-trimethylated amino acid, existing in zwitterionic form at neutral pH. It is now often called glycine betaine to distinguish it from other betaines that are widely distributed in microorganisms, plants and animals. Many naturally occurring betaines serve as organic osmolytes, substances synthesized or taken up from the environment by cells for protection against osmotic stress, drought, high salinity or high temperature. Intracellular accumulation of betaines permits water retention in cells, thus protecting from the effects of dehydration (Wikipedia). Betaine functions as a methyl donor in that it carries and donates methyl functional groups to facilitate necessary chemical processes. In particular it methylates homocysteine to methionine, also producing N,N-dimethylglycine. 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 comes from either the diet or by the oxidation of choline. Betaine insufficiency is associated with the metabolic syndrome, lipid disorders and diabetes, and may have a role in vascular and other diseases (PMID: 20346934 Link_out). Betaine is important in development, from the pre-implantation embryo to infancy. Betaine is also widely regarded as an anti-oxidant. Betaine has been shown to have an inhibitory effect on NO release in activated microglial cells and may be an effective therapeutic component to control neurological disorders (PMID: 22801281 Link_out). 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 | PDB | 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 Reference
Blood Detected and Quantified
33.6 (23.9-42.1) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
82.0 (20.0-144.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
72 +/- 22.4 uM Adult (>18 years old) Not Specified Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Normal
Urine Detected and Quantified
11.5 (2.7-24.7) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
4.14 umol/mmol creatinine Adult (>18 years old) Male Normal
  • Shaykhutdino...
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected and Quantified
6.1 +/- 3.2 umol/mmol creatinine Adult (>18 years old) Male Normal
Urine Detected and Quantified
49.6 (6.4-92.7) umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 42.1 +/- 19.3 uM Adult (>18 years old) Not Specified Heart Transplant
Blood Detected and Quantified 32.5 (23.9-42.5) uM Adult (>18 years old) Both Chronic renal failure
Blood Detected and Quantified 25.6 (23.4-36.6) uM Adult (>18 years old) Both Continuous ambulatory peritoneal dialysis
Blood Detected and Quantified 31.9 (28.3-36.2) uM Adult (>18 years old) Both Hemodialysis
Urine Detected and Quantified 6.0 (0.0-16.0) umol/mmol creatinine Adult (>18 years old) Both Lung cancer
Urine Detected and Quantified 51.2 +/- 7.6 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency
Urine Detected and Quantified 76.2 +/- 8 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency
Urine Detected and Quantified 210.1 +/- 16.4 umol/mmol creatinine Adult (>18 years old) Both Arginosuccinic aciduria
Urine Detected and Quantified 20.1 +/- 4.7 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia
Urine Detected and Quantified 122 +/- 13.6 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia
Urine Detected and Quantified 8.5 +/- 0.4 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria
Urine Detected and Quantified 14.3 +/- 2.7 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria
Urine Detected and Quantified 14.2 +/- 0.9 umol/mmol creatinine Adult (>18 years old) Both Maple syrup urine disease
Urine Detected and Quantified 58 +/- 2.2 umol/mmol creatinine Adult (>18 years old) Both Aminoaciduria
Associated Disorders and Diseases
Disease References
Lung Cancer
  • Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024 Link_out
      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
                  DrugBank Metabolite 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
                  14. Lever M, Slow S: The clinical significance of betaine, an osmolyte with a key role in methyl group metabolism. Clin Biochem. 2010 Jun;43(9):732-44. doi: 10.1016/j.clinbiochem.2010.03.009. Epub 2010 Mar 25. Pubmed: 20346934 Link_out
                  15. Amiraslani B, Sabouni F, Abbasi S, Nazem H, Sabet M: Recognition of betaine as an inhibitor of lipopolysaccharide-induced nitric oxide production in activated microglial cells. Iran Biomed J. 2012;16(2):84-9. Pubmed: 22801281 Link_out

                  Enzymes
                  Name: Choline dehydrogenase, mitochondrial
                  Reactions: Not Available
                  Gene Name: CHDH
                  Uniprot ID: Q8NE62 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Betaine--homocysteine S-methyltransferase 1
                  Reactions: Not Available
                  Gene Name: BHMT
                  Uniprot ID: Q93088 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Sodium- and chloride-dependent betaine transporter
                  Reactions: Not Available
                  Gene Name: SLC6A12
                  Uniprot ID: P48065 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: S-methylmethionine--homocysteine S-methyltransferase BHMT2
                  Reactions: Not Available
                  Gene Name: BHMT2
                  Uniprot ID: Q9H2M3 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA