Betaine
Betaine is a methylating agent used to treat homocystinuria, a group of genetic disorders characterized by dangerously elevated levels of homocysteine in the blood. It works by donating methyl groups to help convert homocysteine into methionine, thereby reducing homocysteine levels and preventing associated complications such as intellectual disability, skeletal abnormalities, and thromboembolic events.
Betaine is a supplement that lowers elevated homocysteine levels in patients with homocystinuria by facilitating the conversion of homocysteine to methionine.
Key facts at a glance
- ATC
- A16AA02
- RxNorm
- unknown
- Generic
- Betaine
- Class
- atc-a16
- Brands
- Cystadane
- Availability
- otc
- Pregnancy
- B
- Lactation
- safe
- Half-life (h)
- 3
- Onset (min)
- 0
- Duration (h)
- 0
- Routes
- oral
- Forms
- tablet, capsule, sachet, powder
- Regions
- United States, European Union, Canada, Australia
- Monitoring
- homocysteine levels, kidney function
This page is for informational purposes only.
Do not use this information to diagnose yourself or to replace a physician's advice. Always consult a qualified healthcare provider before starting, stopping, or changing any medication. If you believe you are experiencing a medical emergency, call your local emergency number immediately.
Dosage and administration
- Usual dose
- 3g twice daily
- Maximum
- consult physician
- Frequency
- every 12 hours
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
- Usual dose
- 1.5g twice daily
- Maximum
- consult physician
- Frequency
- every 12 hours
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
- Usual dose
- 3g twice daily
- Maximum
- consult physician
- Frequency
- every 12 hours
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
Overdose information
Special Populations
10 groupspregnancy
first trimesteravoid if possible
use only if benefits outweigh potential risks to fetus
regular prenatal monitoring
Use with caution and monitor for adverse effects.
pregnancy
second trimesteruse lowest effective dose
continue only if needed for maternal condition
routine prenatal care
Use with caution and monitor for adverse effects.
pregnancy
third trimestermaintain current dose
do not discontinue without medical advice
fetal monitoring
Use with caution and monitor for adverse effects.
lactation
breastfeedinguse with caution
breastfeeding mothers should consult physician
monitor infant for adverse effects
pediatric_age
children 2 11 yearsweight-based dosing
pediatric formulation available for homocystinuria treatment
regular plasma homocysteine monitoring
pediatric_age
adolescents 12 17 yearsadult dosing may apply
follow adult dosing guidelines
periodic homocysteine level checks
geriatric
elderly 65 years and olderno specific adjustment needed
assess renal function before initiating therapy
monitor renal function
Use with caution and monitor for adverse effects.
renal_impairment
mild renal impairmentno dose adjustment typically required
monitor renal function periodically
monitor creatinine clearance
Use with caution and monitor for adverse effects.
renal_impairment
severe renal impairmentconsider dose reduction
use with caution and monitor closely
frequent renal function monitoring
Use with caution and monitor for adverse effects.
hepatic_impairment
mild to moderate hepatic impairmentno specific guidelines
use with caution in hepatic impairment
monitor liver function tests
Use with caution and monitor for adverse effects.
Statistics and clinical data
FDA approval year
Betaine (Cystadane) FDA approval for homocystinuria treatment
Drug class
Classification as methyl donor compound
Primary indication
Primary therapeutic use for genetic metabolic disorder
Mechanism of action
Pharmacological mechanism of action
Treatment of homocystinuria, including deficiency of cystathionine beta-synthase and remethylation defects, where elevated homocysteine levels cause risk of serious complications.
Betaine acts as a methyl group donor in the remethylation pathway, enabling the conversion of homocysteine to methionine, thereby lowering blood homocysteine concentrations.
Treatment is typically lifelong and requires regular monitoring of homocysteine levels. Should be used as part of a comprehensive management plan including dietary modifications. Adverse effects are generally mild and uncommon.
Contraindications
Conditions and patient states where this medication should be avoided or used with caution.
Do not use (1)
Known allergy to this medication or its components
Use caution (1)
Consult healthcare provider for benefits vs risks assessment
Official drug label
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This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy