Identification

Name
Glycine betaine
Accession Number
DB06756
Type
Small Molecule
Groups
Approved, Nutraceutical
Description

Betaine is a methyl group donor that functions in the normal metabolic cycle of methionine and reduces homocystinuria in patients with inborn errors of methionine metabolism. In the United States, betaine is distributed under the brand name Cystadane® by Rare Disease Therapeutics. Many reports have shown that betaine's therapeutic effectiveness is limited, and does not lower tHcy levels or prevent clinical symptoms [2].

Structure
Thumb
Synonyms
  • 2-(Trimethylammonio)Acetate
  • 2-N,N,N-trimethylammonio acetate
  • 2-trimethylammonioacetate
  • abromine
  • acidol
  • Betaine
  • Betaine, anhydrous
  • Glycinebetaine
  • N,N,N-trimethylammonioacetate
  • N,N,N-Trimethylglycine
  • TMG
  • Trimethylaminoacetate
  • Trimethylammonioacetate
  • Trimethylglycine
  • trimethylglycocoll
External IDs
FEMA NO. 4223 / NSC-166511
Product Ingredients
IngredientUNIICASInChI Key
Betaine citrate19JX795NXV17671-50-0YKXUOESQDCXGIW-UHFFFAOYSA-M
Betaine hydrochlorideJK8U8K4D6K590-46-5HOPSCVCBEOCPJZ-UHFFFAOYSA-N
Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
CystadanePowder, for solution1 g/1gOralRopack Inc.2017-04-13Not applicableUs
CystadanePowder, for solution1 gOralRecordati Rare Diseases Canada Inc1998-08-11Not applicableCanada
CystadanePowder1 gOralOrphan Europe S.A.R.L.2007-02-15Not applicableEu
CystadanePowder, for solution6 g/1OralRare Disease Therapeutics, Inc.1996-10-25Not applicableUs
CystadanePowder, for solution1 g/1gOralOrphan Europe Sarl1996-10-25Not applicableUs
CystadanePowder, for solution180 g/1gOralJazz Pharmaceuticals2007-03-31Not applicableUs
Over the Counter Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
ATONO2 Oxygen BabyLotion0.2 g/100gTopicalLaorganic Co., Ltd.2015-09-01Not applicableUs
Betaine HCl Tab 500mgTablet500 mgOralGahler Enterprises Ltd.1991-12-312001-01-04Canada
Dr.g Pore Purifing TonerLiquid0.5 g/100mLTopicalGOWOONSESANG COSMETICS CO., LTD.2015-07-052018-01-09Us
Mixture Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing End
Acididyn TabBetaine hydrochloride (178.2 mg) + Ammonium chloride (32.4 mg)TabletOralNutri Dyn Products Ltd.1975-12-311997-08-22Canada
Alka-pan TabletsBetaine hydrochloride (20 mg) + Bromelains (50 mg) + Ox bile extract (35 mg) + Pancrelipase (135 mg) + Papaya (125 mg)TabletOralMorter HealthsystemNot applicableNot applicableCanada
Bemosin TabBetaine hydrochloride (130 mg) + Ammonium chloride (97.2 mg) + Pepsin (130 mg)TabletOralTherapeutic Foods Co.1988-12-312003-07-16Canada
BetacolBetaine hydrochloride (61 mg) + Calcium glycerophosphate (50 mg) + Choline bitartrate (2.5 mg) + Potassium bicarbonate (41 mg)CapsuleOralTherapeutic Foods Co.1989-12-151996-09-09Canada
Betaine HCl and PepsinBetaine hydrochloride (324 mg) + Pepsin (135 mg)TabletOralRheingold Food International Ltd.1985-12-312007-07-26Canada
Betasin TabBetaine hydrochloride (130 mg) + Ammonium chloride (97.2 mg) + Pepsin (130 mg)TabletOralBio Vita1987-12-311996-09-09Canada
DygestBetaine hydrochloride (90 mg) + Ox bile extract (75 mg) + Pancrelipase (200 mg) + Papain (100 mg) + Peppermint (50 mg) + Pepsin (125 mg)TabletOralCreative Nutrition Canada Corp.1987-12-312007-07-11Canada
Face It Hd Perfect Bb Spf30 Pa 01Glycine betaine (2 mL/100mL) + Octinoxate (6 mL/100mL) + Talc (0.684 mL/100mL) + Titanium dioxide (6.36 mL/100mL) + Zinc oxide (2.88 mL/100mL)CreamTopicalThefaceshop Co., Ltd.2011-08-23Not applicableUs
Face It Hd Perfect Bb Spf30 Pa 02Glycine betaine (2 mL/100mL) + Octinoxate (6 mL/100mL) + Talc (0.684 mL/100mL) + Titanium dioxide (6.36 mL/100mL) + Zinc oxide (2.88 mL/100mL)CreamTopicalThefaceshop Co., Ltd.2011-08-23Not applicableUs
Glutamic Acid HCl Betaine HCl W PepsinBetaine hydrochloride (100 mg) + Glutamic acid hydrochloride (200 mg) + Pepsin (100 mg)TabletOralNu Life Nutrition Ltd.1963-12-312000-03-03Canada
Unapproved/Other Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing End
EnBrace HRGlycine betaine (500 ug/1) + 1,2-docosahexanoyl-sn-glycero-3-phosphoserine calcium (6.4 mg/1) + 1,2-icosapentoyl-sn-glycero-3-phosphoserine calcium (800 ug/1) + Cobamamide (50 ug/1) + Cocarboxylase (25 ug/1) + Ferrous cysteine glycinate (13.6 mg/1) + Flavin adenine dinucleotide (25 ug/1) + Folic Acid (1 mg/1) + Leucovorin (2.5 mg/1) + Levomefolate magnesium (5.23 mg/1) + Magnesium ascorbate (24 mg/1) + NADH (25 ug/1) + Phosphatidyl serine (12 mg/1) + Pyridoxal Phosphate (25 ug/1) + Magnesium L-threonate (1 mg/1) + Zinc ascorbate (1 mg/1)Capsule, delayed release pelletsOralJaymac Pharmaceuticals, Llc2015-04-08Not applicableUs
EnLyteGlycine betaine (500 ug/1) + 1,2-docosahexanoyl-sn-glycero-3-phosphoserine calcium (6.4 mg/1) + 1,2-icosapentoyl-sn-glycero-3-phosphoserine calcium (800 ug/1) + Citric acid monohydrate (1.83 mg/1) + Cobamamide (50 ug/1) + Cocarboxylase (25 ug/1) + Ferrous cysteine glycinate (13.6 mg/1) + Flavin adenine dinucleotide (25 ug/1) + Folic Acid (1 mg/1) + Leucovorin (2.5 mg/1) + Levomefolate magnesium (7 mg/1) + Magnesium ascorbate (24 mg/1) + NADH (25 ug/1) + Phosphatidyl serine (12 mg/1) + Pyridoxal Phosphate (25 ug/1) + Sodium Citrate (3.67 mg/1) + Magnesium L-threonate (1 mg/1) + Zinc ascorbate (1 mg/1)Capsule, delayed release pelletsOralJaymac Pharmaceuticals Llc2011-10-01Not applicableUs
Categories
UNII
3SCV180C9W
CAS number
107-43-7
Weight
Average: 117.1463
Monoisotopic: 117.078978601
Chemical Formula
C5H11NO2
InChI Key
KWIUHFFTVRNATP-UHFFFAOYSA-N
InChI
InChI=1S/C5H11NO2/c1-6(2,3)4-5(7)8/h4H2,1-3H3
IUPAC Name
2-(trimethylazaniumyl)acetate
SMILES
C[N+](C)(C)CC([O-])=O

Pharmacology

Indication

Betaine is indicated for the treatment of homocystinuria to decrease elevated homocysteine blood levels. Included within the category of homocystinuria are deficiencies or defects in: 1. cystathionine beta-synthase (CBS), 2. 5,10-methylenetetrahydrofolate reductase (MTHFR), 3. cobalamin cofactor metabolism (cbl).

Associated Conditions
Pharmacodynamics
Not Available
Mechanism of action

Betaine acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria.

TargetActionsOrganism
UApoptosis regulator Bcl-2Not AvailableHuman
Absorption

After a single oral dose of betaine (50 mg/kg), absorption was rapid (tmax = 0.9 ± 0.3 hours and a Cmax = 0.9 ± 0.2 mM).

Volume of distribution

V/F = 1.3 l/kg

Protein binding
Not Available
Metabolism
Not Available
Route of elimination
Not Available
Half life

14 hours

Clearance

84 ml/h/kg

Toxicity

In an acute toxicology study in rats, death frequently occurred at doses equal to or greater than 10,000 mg/kg.

Affected organisms
  • Humans and other mammals
Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
Not Available
Food Interactions
Not Available

References

General References
  1. Diekman EF, de Koning TJ, Verhoeven-Duif NM, Rovers MM, van Hasselt PM: Survival and psychomotor development with early betaine treatment in patients with severe methylenetetrahydrofolate reductase deficiency. JAMA Neurol. 2014 Feb;71(2):188-94. doi: 10.1001/jamaneurol.2013.4915. [PubMed:24323041]
  2. Yokoi K, Ito T, Ohkubo Y, Sumi S, Ueta A, Sugiyama N, Togari H: Long follow up of betaine therapy in two Japanese siblings with cystathionine beta-synthase deficiency. Pediatr Int. 2008 Oct;50(5):694-5. doi: 10.1111/j.1442-200X.2008.02717.x. [PubMed:19261122]
External Links
Human Metabolome Database
HMDB0000043
KEGG Drug
D07523
KEGG Compound
C00719
PubChem Compound
247
PubChem Substance
310264879
ChemSpider
242
BindingDB
50103520
ChEBI
17750
ChEMBL
CHEMBL1182
Drugs.com
Drugs.com Drug Page
Wikipedia
Trimethylglycine
ATC Codes
A09AB02 — Betaine hydrochlorideA16AA06 — Betaine
AHFS Codes
  • 56:16.00 — Digestants
  • 92:92.00 — Other Miscellaneous Therapeutic Agents
FDA label
Download (35.7 KB)
MSDS
Download (47.6 KB)

Clinical Trials

Clinical Trials
PhaseStatusPurposeConditionsCount
0TerminatedTreatmentChronic Hepatitis C Virus (HCV) Infection / Genotype 1 / Relapses1
2Active Not RecruitingOtherBMI >30 kg/m2 / Dysglycaemia1
2CompletedTreatmentHomocystinuria1
2CompletedTreatmentHyperoxaluria1
2CompletedTreatmentNonalcoholic Steatohepatitis1
2, 3CompletedTreatmentHepatitis C Viral Infection1
3CompletedTreatmentPeroxisome Biogenesis Disorder (PBD)1
4CompletedPreventionArsenic Metabolites Measured in Urine1
Not AvailableCompletedTreatmentAngelman's syndrome / Nervous System Diseases1
Not AvailableWithdrawnTreatmentChronic Hepatitis C Virus (HCV) Infection1

Pharmacoeconomics

Manufacturers
Not Available
Packagers
Not Available
Dosage forms
FormRouteStrength
TabletOral
LotionTopical0.2 g/100g
CapsuleOral
TabletOral500 mg
PowderOral1 g
Powder, for solutionOral1 g
Powder, for solutionOral1 g/1g
Powder, for solutionOral180 g/1g
Powder, for solutionOral6 g/1
LiquidTopical0.5 g/100mL
Capsule, delayed release pelletsOral
CreamTopical
Kit
PatchTopical
Prices
Not Available
Patents
Not Available

Properties

State
Solid
Experimental Properties
Not Available
Predicted Properties
PropertyValueSource
Water Solubility1.86 mg/mLALOGPS
logP-2.7ALOGPS
logP-4.5ChemAxon
logS-2ALOGPS
pKa (Strongest Acidic)2.26ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area40.13 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity52.82 m3·mol-1ChemAxon
Polarizability12.11 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted ADMET features
Not Available

Spectra

Mass Spec (NIST)
Not Available
Spectra
SpectrumSpectrum TypeSplash Key
Predicted GC-MS Spectrum - GC-MSPredicted GC-MSNot Available
Mass Spectrum (Electron Ionization)MSsplash10-0a4l-9000000000-f32989f795eeceb8b692
MS/MS Spectrum - Quattro_QQQ 10V, PositiveLC-MS/MSsplash10-014i-0900000000-feacc600820771d2a77b
MS/MS Spectrum - Quattro_QQQ 25V, PositiveLC-MS/MSsplash10-0a4i-9000000000-e5bbda9fb66994576062
MS/MS Spectrum - Quattro_QQQ 40V, PositiveLC-MS/MSsplash10-0a4i-9000000000-c382759eab7d0a87ac27
LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, PositiveLC-MS/MSsplash10-014i-0900000000-a7beb42e6944181ae5d9
LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, PositiveLC-MS/MSsplash10-014i-1900000000-77fbc4a2b76030d53644
LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, PositiveLC-MS/MSsplash10-0a4i-9100000000-2941e4997f202293bf71
LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, PositiveLC-MS/MSsplash10-0a4i-9000000000-9b9e3cecb77ed4b40b7c
LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, PositiveLC-MS/MSsplash10-0a4i-9000000000-27bbb5f9642306216a0b
LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , PositiveLC-MS/MSsplash10-014i-1900000000-82a41cbb0f697910b577
LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, PositiveLC-MS/MSsplash10-0aor-9500000000-9a0f1853188052d798c6
Predicted MS/MS Spectrum - 10V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Positive (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 10V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 20V, Negative (Annotated)Predicted LC-MS/MSNot Available
Predicted MS/MS Spectrum - 40V, Negative (Annotated)Predicted LC-MS/MSNot Available
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-066r-5900000000-a6291f86046f32ad3c81
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0a4i-9200000000-709208d6782f1b588501
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-014i-0900000000-a7beb42e6944181ae5d9
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-014i-1900000000-77fbc4a2b76030d53644
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0a4i-9100000000-2941e4997f202293bf71
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0a4i-9000000000-a2560fc2add75f79eadb
LC-MS/MS Spectrum - LC-ESI-QQ , positiveLC-MS/MSsplash10-0a4i-9000000000-27bbb5f9642306216a0b
LC-MS/MS Spectrum - LC-ESI-ITFT , positiveLC-MS/MSsplash10-014i-0900000000-b45353825d98488d752a
LC-MS/MS Spectrum - LC-ESI-IT , positiveLC-MS/MSsplash10-0aor-9500000000-3fdd9d17a169db5c58dd
LC-MS/MS Spectrum - LC-ESI-QTOF , positiveLC-MS/MSsplash10-014i-1900000000-82a41cbb0f697910b577
LC-MS/MS Spectrum - LC-ESI-QTOF , positiveLC-MS/MSsplash10-0aor-9500000000-9a0f1853188052d798c6
MS/MS Spectrum - , positiveLC-MS/MSsplash10-014i-0900000000-fe34051d8d2d4fed6de5
1H NMR Spectrum1D NMRNot Applicable
13C NMR Spectrum1D NMRNot Applicable
[1H,1H] 2D NMR Spectrum2D NMRNot Applicable
[1H,13C] 2D NMR Spectrum2D NMRNot Applicable

Taxonomy

Description
This compound belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon).
Kingdom
Organic compounds
Super Class
Organic acids and derivatives
Class
Carboxylic acids and derivatives
Sub Class
Amino acids, peptides, and analogues
Direct Parent
Alpha amino acids
Alternative Parents
Tetraalkylammonium salts / Carboxylic acid salts / Monocarboxylic acids and derivatives / Carboxylic acids / Organopnictogen compounds / Organic salts / Organic oxides / Hydrocarbon derivatives / Carbonyl compounds / Amines
Substituents
Alpha-amino acid / Quaternary ammonium salt / Tetraalkylammonium salt / Carboxylic acid salt / Monocarboxylic acid or derivatives / Carboxylic acid / Organopnictogen compound / Hydrocarbon derivative / Organic salt / Organic oxygen compound
Molecular Framework
Aliphatic acyclic compounds
External Descriptors
glycine derivative, amino-acid betaine (CHEBI:17750) / a methylated methyl acceptor (BETAINE)

Targets

Kind
Protein
Organism
Human
Pharmacological action
Unknown
Curator comments
Results in downregulation of the target.
General Function
Ubiquitin protein ligase binding
Specific Function
Suppresses apoptosis in a variety of cell systems including factor-dependent lymphohematopoietic and neural cells. Regulates cell death by controlling the mitochondrial membrane permeability. Appea...
Gene Name
BCL2
Uniprot ID
P10415
Uniprot Name
Apoptosis regulator Bcl-2
Molecular Weight
26265.66 Da
References
  1. Kennedy RK, Naik PR, Veena V, Lakshmi BS, Lakshmi P, Krishna R, Sakthivel N: 5-Methyl phenazine-1-carboxylic acid: a novel bioactive metabolite by a rhizosphere soil bacterium that exhibits potent antimicrobial and anticancer activities. Chem Biol Interact. 2015 Apr 25;231:71-82. doi: 10.1016/j.cbi.2015.03.002. Epub 2015 Mar 9. [PubMed:25765238]

Enzymes

Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Inhibitor
General Function
Identical protein binding
Specific Function
Esterase with broad substrate specificity. Contributes to the inactivation of the neurotransmitter acetylcholine. Can degrade neurotoxic organophosphate esters.
Gene Name
BCHE
Uniprot ID
P06276
Uniprot Name
Cholinesterase
Molecular Weight
68417.575 Da
References
  1. Zhukovskii IuG, Kuznetsova LP, Sochilina EE, Dmitrieva EN, Gololobov IuG, Bykovskaia EIu: [Inhibition of cholinesterases of varying origin by ordinary and betaine vinylphosphates]. Ukr Biokhim Zh (1978). 1996 Sep-Oct;68(5):15-20. [PubMed:9229847]
  2. Lisa TA, Garrido MN, Domenech CE: Induction of acid phosphatase and cholinesterase activities in Ps. aeruginosa and their in-vitro control by choline, acetylcholine and betaine. Mol Cell Biochem. 1983;50(2):149-55. [PubMed:6406829]
Kind
Protein
Organism
Human
Pharmacological action
Unknown
Actions
Inhibitor
General Function
Proline dehydrogenase activity
Specific Function
Converts proline to delta-1-pyrroline-5-carboxylate.
Gene Name
PRODH
Uniprot ID
O43272
Uniprot Name
Proline dehydrogenase 1, mitochondrial
Molecular Weight
68001.405 Da
References
  1. Jaleel CA, Manivannan P, Sankar B, Kishorekumar A, Panneerselvam R: Calcium chloride effects on salinity-induced oxidative stress, proline metabolism and indole alkaloid accumulation in Catharanthus roseus. C R Biol. 2007 Sep;330(9):674-83. Epub 2007 Aug 10. [PubMed:17720584]
  2. Jaleel CA, Manivannan P, Sankar B, Kishorekumar A, Gopi R, Somasundaram R, Panneerselvam R: Water deficit stress mitigation by calcium chloride in Catharanthus roseus: effects on oxidative stress, proline metabolism and indole alkaloid accumulation. Colloids Surf B Biointerfaces. 2007 Oct 15;60(1):110-6. Epub 2007 Jun 14. [PubMed:17643271]
  3. Jaleel CA, Manivannan P, Lakshmanan GM, Sridharan R, Panneerselvam R: NaCl as a physiological modulator of proline metabolism and antioxidant potential in Phyllanthus amarus. C R Biol. 2007 Nov;330(11):806-13. Epub 2007 Sep 25. [PubMed:17923374]
  4. Jaleel CA, Manivannan P, Kishorekumar A, Sankar B, Gopi R, Somasundaram R, Panneerselvam R: Alterations in osmoregulation, antioxidant enzymes and indole alkaloid levels in Catharanthus roseus exposed to water deficit. Colloids Surf B Biointerfaces. 2007 Oct 1;59(2):150-7. Epub 2007 May 6. [PubMed:17560094]

Drug created on September 14, 2010 10:21 / Updated on October 21, 2018 20:34