Idebenone
Pronunciation: (eye-DEB-eh-known).
- Generic name
- idebenone
- Brand names
- Ideben, Neurox, Raxone, Idenone, Catena
- Drug class
- General Medical Info
Availability and supply rules vary by country
Trade names by country: United States
Australia: · Full directory
What idebenone is and how it works
Uses, formulations, monitoring, and supply rules can differ by patient, product, and country. Follow the exact local product information and professional advice.
Mechanism of action: how idebenone works
Idebenone is a synthetic short-chain benzoquinone structurally related to coenzyme Q10. It acts as an electron carrier in the mitochondrial respiratory chain, bypassing complex I defects and restoring cellular ATP production. Its antioxidant properties help reduce oxidative stress and protect neuronal cells from free radical-induced damage, particularly in mitochondrial disorders such as LHON.
What idebenone is used for
Friedreich's ataxia
Investigational/off-label use only — idebenone is not an approved treatment for Friedreich's ataxia in the countries where it is approved (its approved indication is LHON); any use here would be specialist-directed and outside standard labeling. (2)
Leber's hereditary optic neuropathy (LHON)
The approved indication for idebenone (brand name Raxone) in the European Union, UK, Switzerland, and several other countries, for adolescent and adult patients with visual impairment from LHON. Not FDA-approved in the United States as of August 2026. (2)
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome
Investigational/off-label use only — idebenone is not an approved treatment for MELAS; any use here would be specialist-directed and outside standard labeling. (2)
Before taking idebenone
Do not take / not appropriate for
- Do not take idebenone (Raxone) if you are hypersensitive to idebenone or to any of the other ingredients of the tablet. This is the only absolute contraindication in the EU Summary of Product Characteristics. (European Union / European Economic Area)
- Raxone tablets contain lactose. Patients with rare hereditary problems of galactose intolerance, total lactase deficiency, or glucose-galactose malabsorption should not take Raxone. (European Union / European Economic Area)
- Idebenone is not approved by the US Food and Drug Administration for any medical use, so there is no US prescribing information and no US-authorised medicine to take. Products sold in the United States containing idebenone are cosmetics or supplements, not the licensed medicine described here. (United States)
Tell your clinician about
- Any allergy to idebenone or to tablet ingredients, and any intolerance of lactose or of the colouring sunset yellow (E110), which may cause allergic reactions.
- Any liver or kidney problems — caution is advised, and liver problems have led to treatment being interrupted or stopped.
- If you are pregnant, could become pregnant, or are planning a pregnancy — safety in pregnancy has not been established, and idebenone should only be used if the benefit is judged to outweigh the potential risk.
- If you are breastfeeding — idebenone passes into milk in animals and a risk to the infant cannot be excluded, so you and your doctor must decide whether to stop breastfeeding or stop treatment.
- Every other medicine you take, especially digoxin, dabigatran, aliskiren, ciclosporin, tacrolimus, sirolimus, fentanyl, quinidine, pimozide or an ergot medicine.
- If your urine turns reddish-brown — this chromaturia is a harmless effect of idebenone, but tell your doctor so that other causes are not missed.
How to take idebenone
- General Instructions: Take idebenone exactly as prescribed by your doctor. Take it WITH FOOD — food increases how much idebenone your body absorbs by roughly 5 to 7 times, so taking it on an empty stomach can make it much less effective.. Adult Dosing: The approved dose for LHON is 900 mg per day, divided into three doses of 300 mg each.. Pediatric Dosing: For adolescents aged 12 years and older, the dosing is generally the same as adults. Safety and efficacy in children under 12 have not been established.. Administration: Swallow tablets whole with a glass of water, with food. Do not crush or chew the tablets.. When To Stop: Do not stop taking idebenone without consulting your doctor, even if you feel better. Stopping suddenly may reduce its effectiveness.. Storage: Store at room temperature (below 25°C/77°F), away from moisture and heat; the manufacturer states no special storage conditions are required. Keep out of reach of children.
- This page does not provide a dose calculator. Dosing must follow the prescriber, pharmacist, or product label.
Procedures and treatment changes
Tell the treating team before surgery, procedures, or any medicine change. Do not alter treatment without the responsible clinician. (2)
Missed dose
If you miss a dose, take it as soon as you remember. If it is almost time for your next dose, skip the missed dose and resume your regular schedule. Do not double the dose to catch up.
Overdose
If you or someone else takes too much idebenone, call your local poison control center or seek emergency medical attention immediately. Symptoms of overdose may include severe gastrointestinal upset or liver dysfunction. (2)
Side effects
Side effects and their urgency vary; report severe, persistent, or concerning symptoms to a healthcare professional.
Diarrhea
Incidence: Reported in the clinician-authored database; frequency not established
Nausea
Incidence: Reported in the clinician-authored database; frequency not established
Abdominal pain
Incidence: Reported in the clinician-authored database; frequency not established
Rash
Incidence: Reported in the clinician-authored database; frequency not established
Cough
Incidence: Reported in the clinician-authored database; frequency not established
Sore throat
Incidence: Reported in the clinician-authored database; frequency not established
Increased liver enzymes
Incidence: Reported in the clinician-authored database; frequency not established
Nasopharyngitis (runny/stuffy nose, sore throat)
Incidence: Very common (per EU SmPC)
Back pain
Incidence: Common (per EU SmPC)
Signs of liver problems (yellowing skin/eyes, dark urine, unusual fatigue, abdominal pain) or signs of a hypersensitivity reaction (rash, swelling, difficulty breathing)
Incidence: Uncommon but should be evaluated
Serious warnings
May cause liver enzyme elevations — serious
Symptoms: May cause liver enzyme elevations; monitor liver function regularly. Use with caution in patients with hepatic impairment. Safety in pregnancy and breastfeeding has not been established. Not recommended for children under 12 years unless specifically prescribed. May interact with other medications metabolized by the liver.
Action: Follow the urgency stated and seek prompt or emergency care when indicated. (2)
Contraindicated in patients with known hypersensitivity to idebenone or any excipient, including the rare hereditary conditions of galactose intolerance, lactase deficiency, or glucose-galactose malabsorption (relevant because of excipients used in the tablet formulation) — serious
Symptoms: Contraindicated in patients with known hypersensitivity to idebenone or any excipient, including the rare hereditary conditions of galactose intolerance, lactase deficiency, or glucose-galactose malabsorption (relevant because of excipients used in the tablet formulation). Use with caution in patients with hepatic or renal impairment, since adverse events in these groups have led to temporary interruption or discontinuation of therapy. Safety in pregnancy and lactation is not established; use only if benefits outweigh risks. Monitor for signs of hypersensitivity and liver dysfunction.
Action: Follow the urgency stated and seek prompt or emergency care when indicated. (2)
Interactions
| Medicine or test | Effect | Action |
|---|---|---|
| Recorded interactions | Idebenone may interact with drugs that affect liver enzymes, such as rifampicin, phenytoin, or carbamazepine. It may also interact with certain herbal supplements and over-the-counter medications. Always inform your healthcare provider about all medicines and supplements you are taking to avoid potential interactions. |
Pregnancy, breastfeeding, kidney/liver function, age
Pregnancy
There is no established human safety data for idebenone in pregnancy. Treatment during pregnancy requires the prescribing clinician to individually weigh the potential benefit against the potential risk. (2)
Breastfeeding
Idebenone is excreted into breast milk. The decision to stop breastfeeding or to stop idebenone therapy should be made individually, weighing the benefit of breastfeeding for the child against the benefit of therapy for the mother, in discussion with the treating clinician. (2)
Children
Frequency: Not applicable. Weight Based: Not recommended for pediatric use. Max Daily Dose: Not applicable. Max Single Dose: Not applicable. Age Restrictions: Safety not established in children (2)
Renal_impairment
Caution is advised in both hepatic and renal impairment: adverse events in these patients have led to temporary interruption or discontinuation of idebenone therapy in clinical experience, and specific renal-impairment dosing data are insufficient. The prescribing clinician monitors more closely in these situations. (2)
Hepatic_impairment
Caution is advised in both hepatic and renal impairment: adverse events in these patients have led to temporary interruption or discontinuation of idebenone therapy in clinical experience, and specific renal-impairment dosing data are insufficient. The prescribing clinician monitors more closely in these situations. (2)
Monitoring
(2)Storage
Store at room temperature (below 25°C/77°F), away from moisture and heat; the manufacturer states no special storage conditions are required beyond this. Keep out of reach of children.
Professional information
Clinician-facing context only. This section does not replace current product labeling.
EU/UK and other countries where Raxone is approved
Indication: Leber's hereditary optic neuropathy (LHON)
Dose summary: 900 mg per day, divided into three doses of 300 mg each, taken with food. (Dosing studied for Friedreich's ataxia and MELAS in research settings has varied and is not an approved indication for idebenone in most countries; any such use is investigational/off-label and specialist-directed.)
Renal context: Specific renal-impairment dosing data are insufficient; caution is advised, and adverse events in patients with renal impairment have led to temporary interruption or discontinuation of therapy in clinical experience. (2)
Frequently asked questions
What is idebenone used for?
Idebenone is mainly used to treat Leber’s Hereditary Optic Neuropathy (LHON), a rare genetic eye disease that causes vision loss. It is also being studied for other mitochondrial and neurodegenerative disorders.
How does idebenone work?
Idebenone works as an antioxidant and supports mitochondrial function, helping protect nerve cells from damage and improving energy production in cells.
What are the most common side effects?
The most common side effects are mild diarrhea, cough, back pain, headache, and abdominal pain.
Can idebenone be used in children?
Where idebenone (Raxone) is approved for LHON (the EU, UK, and several other countries — not the US as of August 2026), it is approved for adolescents aged 12 and older as well as adults. Its safety and effectiveness in children under 12 have not been established.
Is idebenone safe during pregnancy or breastfeeding?
There is limited information about the safety of idebenone during pregnancy or breastfeeding. It should only be used if clearly needed and prescribed by your doctor.
What should I do if I miss a dose?
Take the missed dose as soon as you remember, unless it is almost time for your next dose. Do not double up doses.
Are there any foods or medicines I should avoid while taking idebenone?
The opposite of avoiding food is true: idebenone should be taken WITH food, because food increases how much of the medicine your body absorbs by roughly 5 to 7 times — taking it on an empty stomach can make it much less effective. Discuss alcohol use and any new medications or supplements with your doctor, since idebenone can interact with drugs that affect liver enzymes.
What happens if I take too much Idebenone?
Taking too much Idebenone may lead to an exacerbation of common side effects such as nausea, diarrhea, or headache. In severe cases, it could potentially cause more serious adverse reactions. If you suspect an overdose, it is crucial to seek immediate emergency medical attention. Do not attempt to self-treat or wait for symptoms to worsen.
Can I drink alcohol while taking Idebenone?
While there are no specific contraindications against alcohol consumption with Idebenone, it is generally advisable to exercise caution. As a mitochondrial enhancer, Idebenone aims to improve cellular function. Alcohol can have detrimental effects on cellular health and may counteract the therapeutic benefits of Idebenone. Consult your healthcare provider for personalized advice regarding alcohol intake.
Is Idebenone safe during pregnancy?
Idebenone's pregnancy category requires consultation with a doctor. There is insufficient data to definitively establish its safety during pregnancy. Therefore, it is strongly recommended that pregnant individuals, or those planning to become pregnant, discuss the potential risks and benefits with their healthcare provider before using Idebenone to ensure the well-being of both mother and fetus.
What is the mechanism of action of idebenone?
Idebenone is a synthetic short-chain benzoquinone structurally related to coenzyme Q10. It acts as an electron carrier in the mitochondrial respiratory chain, bypassing complex I defects and restoring cellular ATP production. Its antioxidant properties help reduce oxidative stress and protect neuronal cells from free radical-induced damage, particularly in mitochondrial disorders such as LHON.
What is the generic name of Idebenone?
The generic name is idebenone. It is sold under brand names including Ideben, Neurox, Raxone, Idenone, Catena.
What class of drug is idebenone?
Idebenone is classed as: General Medical Info.
References
- Idebenone — clinician-authored medicine recordMedGuideGlobal · null · accessed 2026-08-06 · source 1
- Raxone 150 mg tablets — Summary of Product Characteristicselectronic Medicines Compendium (UK) · United Kingdom · accessed 2026-08-06 · source 2
- LEBODY LAB RENEWAL DUAL EFFECT IDEBENONE SERUM (NIACINAMIDE, ADENOSINE) LIQUID [GTG WELLNESS CO., LTD.] — US prescribing information (DailyMed SPL)U.S. National Library of Medicine (DailyMed) · US · accessed 2026-08-14 · source 3
- idebenone — RxNorm normalized drug concept (RXCUI 51296)U.S. National Library of Medicine (RxNorm) · US · accessed 2026-08-14 · source 4
Brand names by country
Single-ingredient idebenone names are shown separately by country. Product availability, strengths, and supply rules can change; combination products are not included here.
Priority countries
United States
- KirkmanKirkman
- HBC Health SolutionsHBC Health Solutions
- LifeLinkLifeLink
- Smart NutritionSmart Nutrition
- SuperSmartSuperSmart