Betaine

Identification

Summary

Betaine is a methyl group donor used for the treatment of homocystinuria to decrease elevated homocysteine blood levels.

Brand Names
Amversio, Cystadane, EnBrace HR, EnLyte
Generic Name
Betaine
DrugBank Accession Number
DB06756
Background

Betaine is a methyl group donor that functions in the normal metabolic cycle of methionine. It is a naturally occurring choline derivative commonly ingested through diet, with a role in regulating cellular hydration and maintaining cell function.3,4 Homocystinuria is an inherited disorder that leads to the accumulation of homocysteine in plasma and urine. Currently, no treatments are available to correct the genetic causes of homocystinuria. However, in order to normalize homocysteine levels, patients can be treated with vitamin B6 (pyridoxine), vitamin B12 (cobalamin), folate and specific diets.7 Betaine reduces plasma homocysteine levels in patients with homocystinuria.5 Although it is present in many food products, the levels found there are insufficient to treat this condition. The FDA and EMA have approved the product Cystadane (betaine anhydrous, oral solution) for the treatment of homocystinuria,5,7 and the EMA has approved the use of Amversio (betaine anhydrous, oral powder).6

Type
Small Molecule
Groups
Approved, Investigational, Nutraceutical
Structure
Weight
Average: 117.1463
Monoisotopic: 117.078978601
Chemical Formula
C5H11NO2
Synonyms
  • 2-(Trimethylammonio)Acetate
  • 2-N,N,N-trimethylammonio acetate
  • 2-trimethylammonioacetate
  • Abromine
  • Acidol
  • Betaína anhidra
  • Betaine
  • Betaine, anhydrous
  • Glycine betaine
  • Glycinebetaine
  • Glykokollbetain
  • N,N,N-trimethylammonioacetate
  • N,N,N-Trimethylglycine
  • TMG
  • Trimethylaminoacetate
  • Trimethylammonioacetate
  • Trimethylglycine
  • Trimethylglycocoll
External IDs
  • FEMA NO. 4223
  • NSC-166511

Pharmacology

Indication

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

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Associated Conditions
Indication TypeIndicationCombined Product DetailsApproval LevelAge GroupPatient CharacteristicsDose Form
Management ofHomocystinuria••••••••••••••••••••
Adjunct therapy in management ofHomocystinuria due to cystathionine beta-synthase deficiency••••••••••••••••••
Adjunct therapy in management ofHomocystinuria due to mthfr deficiency••••••••••••••••••
Adjunct therapy in management ofHomocystinuria due to cobalamin cofactor metabolism (cbl) defect••••••••••••••••••
Contraindications & Blackbox Warnings
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Pharmacodynamics

Betaine decreases plasma homocysteine concentrations in homocystinuria cases caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl).5 The decrease of homocysteine is estimated to be 20-30% of pre-treatment levels. Betaine supplementation in patients with homocystinuria also improves metabolic abnormalities in cerebrospinal fluid.6 Reports have shown that depending on the type of homocystinuria, the therapeutic effectiveness of betaine alone may be limited, insufficient to decrease total homocysteine levels and prevent clinical symptoms. In patients with homocystinuria due to cystathionine beta-synthase (CBS) deficiency, betaine should be used when serum total homocysteine levels remain high despite dietary therapy.1

Patients taking betaine for several years do not show evidence of tolerance. Also, betaine concentrations are not correlated with homocysteine concentrations.5 In patients with MTHFR deficiency and cbl defects, betaine may increase methionine and S-adenosyl methionine (SAM) plasma levels. Patients with CBS deficiency without a dietary restriction of methionine may accumulate excessive amounts of methionine. Clinical data shows that in patients with CBS deficiency, increased plasma methionine levels were associated with cerebral edema.5,6

Mechanism of action

Homocystinuria is a hereditary disorder characterized by high levels of the amino acid homocysteine. This condition can be caused by deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylenetetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (cbl).5 CBS converts homocysteine to cystathionine, and a deficiency in this enzyme can lead to an accumulation of homocysteine. MTHFR is responsible for producing 5-methyltetrahydrofolate, a methyl donor that participates in the conversion of homocysteine back to methionine; therefore, an MTHFR deficiency can also lead to homocystinuria.4 Additionally, a defect in cobalamin (vitamin B12) cofactor metabolism can lead to homocystinuria, since a metabolite of cobalamin (methylcobalamin) promotes the conversion of homocysteine to methionine.3

Betaine transfers a methyl group via the enzyme betaine homocysteine methyl transferase (BHMT), converting homocysteine back into methionine and dimethylglycine (DMG).4,6 In patients with homocystinuria, betaine reduces homocysteine levels and improves health outcomes.3

TargetActionsOrganism
ABetaine--homocysteine S-methyltransferase 1
substrate
Humans
Absorption

Betaine is rapidly absorbed and distributed. In healthy volunteers (n=12) given 50 mg/kg of betaine, the Cmax, tmax and AUC0,∞ were 0.939 mmol/L, 0.90 h and 5.52 mmol⋅h/L, respectively.2 No significant changes in absorption kinetics were observed after repeated betaine administration (100 mg/kg/day for 5 days). The absolute bioavailability of betaine anhydrous has not been determined.6

Volume of distribution

Based on a study done on healthy volunteers (n=12) given 50 mg/kg of betaine, the volume of distribution was 1.3 L/kg.2

Protein binding

Not Available

Metabolism

Betaine is catabolized mainly in the mitochondria of liver and kidney cells. The transmethylation of betaine via betaine homocysteine methyl transferase (BHMT) leads to the formation of dimethylglycine.3

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Route of elimination

Betaine is mainly eliminated by metabolism.2 With a slow elimination rate and assuming 100% bioavailability, the renal clearance of betaine is negligible (5% of total body clearance).6

Half-life

In healthy volunteers (n=12) given 50 mg/kg of betaine, the elimination half-life was 14.38 h.2 In volunteers given 100 mg/kg/day of betaine for 5 days, the distribution half-life was significantly longer, suggesting that the transport and redistribution processes of betaine were saturated.6

Clearance

Based on a study done on healthy volunteers (n=12) given 50 mg/kg of betaine, total oral plasma drug clearance was 0.084 L/h⋅kg.2

Adverse Effects
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Toxicity

Toxicity information regarding betaine is not readily available. Patients experiencing an overdose are at an increased risk of severe adverse effects such as cerebral edema in patients with cystathionine beta-synthase (CBS) deficiency.5 Symptomatic and supportive measures are recommended. In an acute toxicology study in rats, death frequently occurred at doses equal to or greater than 10,000 mg/kg.5 The effects of betaine on long-term carcinogenicity and fertility have not been evaluated. The following tests have not shown evidence of betaine genotoxicity: metaphase analysis of human lymphocytes, bacterial reverse mutation assay, and mouse micronucleus test.5

Pathways
Not Available
Pharmacogenomic Effects/ADRs Browse all" title="About SNP Mediated Effects/ADRs" id="snp-actions-info" class="drug-info-popup" href="javascript:void(0);">
Not Available

Interactions

Drug Interactions Learn More" title="About Drug Interactions" id="structured-interactions-info" class="drug-info-popup" href="javascript:void(0);">
This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
DrugInteraction
AcebutololBetaine may increase the bradycardic activities of Acebutolol.
AcetylcholineThe risk or severity of adverse effects can be increased when Betaine is combined with Acetylcholine.
AclidiniumBetaine may increase the neuromuscular blocking activities of Aclidinium.
AmantadineThe therapeutic efficacy of Amantadine can be decreased when used in combination with Betaine.
AmifampridineThe risk or severity of adverse effects can be increased when Betaine is combined with Amifampridine.
Food Interactions
  • Take with or without food. Oral betaine powder should be mixed with food or a beverage and then consumed.

Products

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Product Ingredients
IngredientUNIICASInChI Key
Betaine citrate19JX795NXV17671-50-0YKXUOESQDCXGIW-UHFFFAOYSA-M
Betaine hydrochlorideJK8U8K4D6K590-46-5HOPSCVCBEOCPJZ-UHFFFAOYSA-N
International/Other Brands
Amversio (SERB SA) / Cystadane (Recordati Rare Diseases Inc.)
Brand Name Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
AmversioPowder1 gOralSerb Sa2022-08-02Not applicableEU flag
CystadanePowder, for solution1 g/1gOralRecordati Rare Diseases1996-10-25Not applicableUS flag
CystadanePowder, for solution6 g/1OralRare Disease Therapeutics, Inc.1996-10-25Not applicableUS flag
CystadanePowder1 gOralRecordati Rare Diseases2016-09-08Not applicableEU flag
CystadanePowder, for solution1 g/1gOralOrphan Europe Sarl2018-01-08Not applicableUS flag
Generic Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
BetainePowder, for solution1 g/1gOralCosette Pharmaceuticals, Inc.2022-11-04Not applicableUS flag
Betaine AnhydrousPowder, for solution1 g/1gOralEton Pharmaceuticals, Inc.2023-02-01Not applicableUS flag
Betaine AnhydrousFor solution1 g/1gOralNovitium Pharma Llc2021-11-23Not applicableUS flag
Betaine Anhydrous For Oral SolutionPowder, for solution1 g/1gOralLukare Medical, Llc2022-02-11Not applicableUS flag
Betaine Anhydrous for Oral SolutionFor solution1 g/1gOralOAKRUM PHARMA, LLC2022-02-032024-04-30US flag
Over the Counter Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
ATONO2 Oxygen BabyLotion0.2 g/100gTopicalLaorganic Co., Ltd.2015-09-01Not applicableUS flag
Betaine HCl Tab 500mgTablet500 mg / tabOralGahler Enterprises Ltd.1991-12-312001-01-04Canada flag
Dr.g Pore Purifing TonerLiquid0.5 g/100mLTopicalGOWOONSESANG COSMETICS CO., LTD.2015-07-052018-01-09US flag
Dr.Jucre Rebirth Activating TonerLiquid0.001 g/100mLTopicalNATURECELL CO., LTD.2022-10-01Not applicableUS flag
ENZYME DIGEST TABLETTabletOralEucogen Pharma Sdn. Bhd.2020-09-08Not applicableMalaysia flag
Mixture Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
Acididyn TabBetaine hydrochloride (178.2 mg) + Ammonium chloride (32.4 mg)TabletOralNutri Dyn Products Ltd.1975-12-311997-08-22Canada flag
Alka-pan TabletsBetaine hydrochloride (20 mg) + Bromelains (50 mg) + Ox bile extract (35 mg) + Pancrelipase (135 mg) + Papaya (125 mg)TabletOralMorter HealthsystemNot applicableNot applicableCanada flag
Bemosin TabBetaine hydrochloride (130 mg) + Ammonium chloride (97.2 mg) + Pepsin (130 mg)TabletOralTherapeutic Foods Co.1988-12-312003-07-16Canada flag
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 flag
Betaine HCl and PepsinBetaine hydrochloride (324 mg) + Pepsin (135 mg)TabletOralRheingold Food International Ltd.1985-12-312007-07-26Canada flag
Unapproved/Other Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
ATONO2 Oxygen BabyBetaine (0.2 g/100g)LotionTopicalLaorganic Co., Ltd.2015-09-01Not applicableUS flag
CystadaneBetaine (1 g/1g)Powder, for solutionOralOrphan Europe Sarl2018-01-08Not applicableUS flag
Dr.g Pore Purifing TonerBetaine (0.5 g/100mL)LiquidTopicalGOWOONSESANG COSMETICS CO., LTD.2015-07-052018-01-09US flag
Dr.Jucre Rebirth Activating TonerBetaine (0.001 g/100mL)LiquidTopicalNATURECELL CO., LTD.2022-10-01Not applicableUS flag
EnBrace HRBetaine (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) + Magnesium L-threonate (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) + Zinc ascorbate (1 mg/1)Capsule, delayed release pelletsOralJaymac Pharma2011-08-12Not applicableUS flag

Categories

ATC Codes
A16AA06 — BetaineA09AB02 — Betaine hydrochloride
Drug Categories
Chemical TaxonomyProvided by Classyfire
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
Aliphatic acyclic compound / Alpha-amino acid / Amine / Carbonyl group / Carboxylic acid / Carboxylic acid salt / Hydrocarbon derivative / Monocarboxylic acid or derivatives / Organic nitrogen compound / Organic oxide
Molecular Framework
Aliphatic acyclic compounds
External Descriptors
glycine derivative, amino-acid betaine (CHEBI:17750) / a methylated methyl acceptor (BETAINE)
Affected organisms
  • Humans and other mammals

Chemical Identifiers

UNII
3SCV180C9W
CAS number
107-43-7
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

References

Synthesis Reference

Shandong Aobo Biotechnology Co., Ltd. (2010). Preparation method of anhydrous betaine (Patent No. CN102557970B). China National Intellectual Property Administration. https://patents.google.com/patent/CN102557970B/en

General References
  1. 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. [Article]
  2. Schwahn BC, Hafner D, Hohlfeld T, Balkenhol N, Laryea MD, Wendel U: Pharmacokinetics of oral betaine in healthy subjects and patients with homocystinuria. Br J Clin Pharmacol. 2003 Jan;55(1):6-13. doi: 10.1046/j.1365-2125.2003.01717.x. [Article]
  3. Craig SA: Betaine in human nutrition. Am J Clin Nutr. 2004 Sep;80(3):539-49. doi: 10.1093/ajcn/80.3.539. [Article]
  4. Truitt C, Hoff WD, Deole R: Health Functionalities of Betaine in Patients With Homocystinuria. Front Nutr. 2021 Sep 9;8:690359. doi: 10.3389/fnut.2021.690359. eCollection 2021. [Article]
  5. FDA Approved Drug Products: CYSTADANE (betaine anhydrous) for oral solution [Link]
  6. EMA Summary of Product Characteristics: Amversio (betaine anhydrous) oral powder [Link]
  7. EMA Scientific Discussion: Cystadane (betaine anhydrous) for oral solution [Link]
Human Metabolome Database
HMDB0000043
KEGG Drug
D07523
KEGG Compound
C00719
PubChem Compound
247
PubChem Substance
310264879
ChemSpider
242
BindingDB
50103520
RxNav
1512
ChEBI
17750
ChEMBL
CHEMBL1182
Drugs.com
Drugs.com Drug Page
Wikipedia
Trimethylglycine

Clinical Trials

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Pharmacoeconomics

Manufacturers
Not Available
Packagers
Not Available
Dosage Forms
FormRouteStrength
LotionTopical0.2 g/100g
CapsuleOral
For solutionOral1 g/1g
TabletOral500 mg / tab
PowderOral1 G
Powder, for solutionOral1 g / 1.7 cc
Powder, for solutionOral1 g/1g
Powder, for solutionOral180 g/1g
Powder, for solutionOral6 g/1
Powder, for solutionOral1 g
LiquidTopical0.5 g/100mL
LiquidTopical0.001 g/100mL
Capsule, liquid filledOral
Capsule, delayed release pelletsOral
TabletOral
CreamTopical
ShampooTopical
TabletOral
KitOral
PatchTopical
Prices
Not Available
Patents
Not Available

Properties

State
Solid
Experimental Properties
PropertyValueSource
melting point (°C)301-305°CEMA Scientific Discussion: Cystadane (betaine anhydrous) for oral solution
water solubilitySolubleEMA Scientific Discussion: Cystadane (betaine anhydrous) for oral solution
pKa1.83Betaine: Chemistry, Analysis, Function and Effects. UK, Royal Society of Chemistry, 2015.
Predicted Properties
PropertyValueSource
Water Solubility1.86 mg/mLALOGPS
logP-2.7ALOGPS
logP-4.5Chemaxon
logS-2ALOGPS
pKa (Strongest Acidic)3.26Chemaxon
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-MSsplash10-05fu-9200000000-96ab6e2136fe7e03b63a
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
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
Predicted 1H NMR Spectrum1D NMRNot Applicable
Predicted 13C NMR Spectrum1D NMRNot Applicable
[1H,1H] 2D NMR Spectrum2D NMRNot Applicable
[1H,13C] 2D NMR Spectrum2D NMRNot Applicable
Chromatographic Properties
Collision Cross Sections (CCS)
AdductCCS Value (Å2)Source typeSource
[M-H]-121.1018183
predicted
DarkChem Lite v0.1.0
[M-H]-121.1197183
predicted
DarkChem Lite v0.1.0
[M-H]-120.1318618
predicted
DarkChem Lite v0.1.0
[M-H]-126.56775
predicted
DeepCCS 1.0 (2019)
[M+H]+129.3875
predicted
DeepCCS 1.0 (2019)
[M+Na]+137.90205
predicted
DeepCCS 1.0 (2019)

Targets

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Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Substrate
General Function
Zinc ion binding
Specific Function
Involved in the regulation of homocysteine metabolism. Converts betaine and homocysteine to dimethylglycine and methionine, respectively. This reaction is also required for the irreversible oxidati...
Gene Name
BHMT
Uniprot ID
Q93088
Uniprot Name
Betaine--homocysteine S-methyltransferase 1
Molecular Weight
44998.205 Da
References
  1. Truitt C, Hoff WD, Deole R: Health Functionalities of Betaine in Patients With Homocystinuria. Front Nutr. 2021 Sep 9;8:690359. doi: 10.3389/fnut.2021.690359. eCollection 2021. [Article]

Enzymes

Kind
Protein
Organism
Humans
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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]

Transporters

Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Substrate
General Function
Neurotransmitter:sodium symporter activity
Specific Function
Transports betaine and GABA. May have a role in regulation of GABAergic transmission in the brain through the reuptake of GABA into presynaptic terminals, as well as in osmotic regulation.
Gene Name
SLC6A12
Uniprot ID
P48065
Uniprot Name
Sodium- and chloride-dependent betaine transporter
Molecular Weight
69367.655 Da
References
  1. Zhu XM, Ong WY: Changes in GABA transporters in the rat hippocampus after kainate-induced neuronal injury: decrease in GAT-1 and GAT-3 but upregulation of betaine/GABA transporter BGT-1. J Neurosci Res. 2004 Aug 1;77(3):402-9. doi: 10.1002/jnr.20171. [Article]

Drug created at September 14, 2010 16:21 / Updated at February 21, 2024 02:33