Identification
- Summary
Idebenone is an analogue of ubiquinone used for the treatment of visual impairment in adolescent and adult patients with Leber’s Hereditary Optic Neuropathy (LHON).
- Brand Names
- Raxone
- Generic Name
- Idebenone
- DrugBank Accession Number
- DB09081
- Background
Idebenone is a synthetic analogue of ubiquinone (also known as Coenzyme Q10), a vital cell antioxidant and essential component of the Electron Transport Chain (ETC). It has been proposed that by interacting with the ETC, idebenone increases ATP production required for mitochondrial function, reduces free radicals, inhibits lipid peroxidation, and consequently protects the lipid membrane and mitochondria from oxidative damage 2. More specifically, idebenone is thought to transfer electrons directly to complex III of the mitochondrial ETC, thereby circumventing complex I and restoring cellular energy (ATP) generation 4.
Due to its ability to reduce oxidative damage and improve ATP production, idebenone was originally investigated for its potential use in Alzheimer's Disease and other cognitivie disorders 3. Lack of improvement in cognitive function halted its production for these conditions, however it continues to be investigated for use in other conditions associated with mitochondrial damage.
Idebenone is currently only indicated for use by the European Medicines Agency (EMA) for the treatment of visual impairment in adolescent and adult patients with Leber’s Hereditary Optic Neuropathy (LHON). LHON is a mitochondrially inherited degeneration of retinal ganglion cells, resulting in acute central vision loss. Due to its biochemical mode of action, it's thought that idebenone may re-activate viable-but-inactive retinal ganglion cells (RGCs) in LHON patients 4. It is not currently approved for use by either the Food and Drug Administration (USA) or Health Canada.
- Type
- Small Molecule
- Groups
- Approved, Investigational
- Structure
Structure for Idebenone (DB09081)
- Weight
- Average: 338.444
Monoisotopic: 338.209324066 - Chemical Formula
- C19H30O5
- Synonyms
- 2-(10-hydroxydecyl)-5,6-dimethoxy-3-methyl-1,4-benzoquinone
- 2-(10-hydroxydecyl)-5,6-dimethoxy-3-methyl-p-benzoquinone
- 2-(10-hydroxydecyl)-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione
- 2,3-dimethoxy-5-methyl-6-(10'-hydroxydecyl)-1,4-benzoquinone
- 6-(10-hydroxydecyl)-2,3-dimethoxy-5-methyl-1,4-benzoquinone
- 6-(10-hydroxydecyl)-2,3-dimethoxy-5-methyl-p-benzoquinone
- idebenona
- idébénone
- Idebenone
- External IDs
- BRN 2001459
- BRN-2001459
- BRN2001459
- CV 2619
- CV-2619
- CV2619
Pharmacology
- Indication
Idebenone is indicated for use by the European Medicines Agency (EMA) for the treatment of visual impairment in adolescent and adult patients with Leber’s Hereditary Optic Neuropathy (LHON). It is not currently approved for use by either the Food and Drug Administration (USA) or Health Canada 4.
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- Contraindications & Blackbox Warnings
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Not Available
- Mechanism of action
Idebenone is a synthetic analogue of ubiquinone (also known as Coenzyme Q10), a vital cell antioxidant and essential component of the Electron Transport Chain (ETC). It has been proposed that by interacting with the ETC, idebenone increases ATP production required for mitochondrial function, reduces free radicals, inhibits lipid peroxidation, and consequently protects the lipid membrane and mitochondria from oxidative damage 2. More specifically, idebenone is thought to transfer electrons directly to complex III of the mitochondrial ETC, thereby circumventing complex I and restoring cellular energy (ATP) generation 4.
- Absorption
After oral administration, idebenone is rapidly absorbed. On repeat dosing, maximum plasma concentrations of idebenone are reached on average within 1 hour (median 0.67 h range: 0.33-2.00 h). Food increases the bioavailability of idebenone by approximately 5-7-fold and i therefore should always be administered with food 4.
- Volume of distribution
Experimental data have shown that idebenone passes the blood-brain barrier and is distributed at significant concentrations in cerebral tissue. Following oral administration pharmacologically relevant concentrations of idebenone are detectable in the aqueous humor of the eye 4.
- Protein binding
Not Available
- Metabolism
Metabolism occurs by means of oxidative shortening of the side chain and by reduction of the quinone ring and conjugation to glucuronides and sulphates. Idebenone shows a high first pass metabolism resulting in conjugates of idebenone (glucuronides and sulphates (IDE-C)) and the Phase I metabolites QS10, QS6, and QS4 as well as their corresponding Phase II metabolites (glucuronides and sulphates (QS10+QS10-C, QS6+QS6-C, QS4+QS4-C)). The main metabolites in plasma are IDE-C and QS4+QS4-C 4.
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- Route of elimination
The main route of elimination is metabolism, with the majority of dose excreted via the kidneys as metabolites. After a single or repeated oral dose of 750 mg of idebenone, QS4+QS4-C were the most prominent idebenone-derived metabolites in urine, representing on average between 49.3% and 68.3% of the total administered dose. QS6+QS6 represented 6.45% to 9.46%, whereas QS10+QS10-C and IDE+IDE-C were close to 1% or below 4.
- Half-life
Not Available
- Clearance
Not Available
- Adverse Effects
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The most commonly reported adverse reactions to idebenone are mild to moderate diarrhoea (usually not requiring the discontinuation of the treatment), nasopharyngitis, cough and back pain Label.
- Pathways
- Not Available
- Pharmacogenomic Effects/ADRs
- Not Available
Interactions
- Drug Interactions
- 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.
Drug Interaction Integrate drug-drug
interactions in your softwareAbacavir Abacavir may decrease the excretion rate of Idebenone which could result in a higher serum level. Aceclofenac Aceclofenac may decrease the excretion rate of Idebenone which could result in a higher serum level. Acemetacin Acemetacin may decrease the excretion rate of Idebenone which could result in a higher serum level. Acetaminophen Acetaminophen may decrease the excretion rate of Idebenone which could result in a higher serum level. Acetazolamide Acetazolamide may increase the excretion rate of Idebenone which could result in a lower serum level and potentially a reduction in efficacy. Acetylsalicylic acid Acetylsalicylic acid may decrease the excretion rate of Idebenone which could result in a higher serum level. Aclidinium Aclidinium may decrease the excretion rate of Idebenone which could result in a higher serum level. Acrivastine Idebenone may decrease the excretion rate of Acrivastine which could result in a higher serum level. Acyclovir Acyclovir may decrease the excretion rate of Idebenone which could result in a higher serum level. Adefovir dipivoxil Adefovir dipivoxil may decrease the excretion rate of Idebenone which could result in a higher serum level. 
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- Take with food.
Products
Drug product information from 10+ global regionsOur datasets provide approved product information including:dosage, form, labeller, route of administration, and marketing period.Access drug product information from over 10 global regions.- International/Other Brands
- Sovrima
- Brand Name Prescription Products
Name Dosage Strength Route Labeller Marketing Start Marketing End Region Image Catena Tablet 150 mg Oral Santhera Pharmaceuticals (Switzerland) Limited 2008-10-15 2013-04-30 Canada 
Raxone Tablet, film coated 150 mg Oral Santhera Pharmaceuticals (Deutschland) Gmb H 2016-09-08 Not applicable EU 
- Mixture Products
Name Ingredients Dosage Route Labeller Marketing Start Marketing End Region Image BERMAXIN 90/10/5 MG FILM TABLET ,FILM TABLET, 28 ADET Idebenone (90 mg) + Donepezil hydrochloride (10 mg) + Memantine hydrochloride (5 mg) Tablet, film coated Oral CELTİS İLAÇ SAN. VE TİC. A.Ş. 2020-08-14 Not applicable Turkey 
BERMAXIN 90/10/5 MG FILM TABLET ,FILM TABLET, 84 ADET Idebenone (90 mg) + Donepezil hydrochloride (10 mg) + Memantine hydrochloride (5 mg) Tablet, film coated Oral CELTİS İLAÇ SAN. VE TİC. A.Ş. 2020-08-14 Not applicable Turkey EVERON 90/5 MG FILM KAPLI TABLET ,100 TABLET Idebenone (90 mg) + Memantine hydrochloride (5 mg) Tablet, coated Oral NEUTEC İLAÇ SAN. TİC. A.Ş. 2020-08-14 Not applicable Turkey EVERON 90/5 MG FILM KAPLI TABLET ,50 TABLET Idebenone (90 mg) + Memantine hydrochloride (5 mg) Tablet, coated Oral NEUTEC İLAÇ SAN. TİC. A.Ş. 2020-08-14 Not applicable Turkey SALPEZIL 90/10 MG FILM KAPLI TABLET ,TABLET, 28 ADET Idebenone (90 mg) + Donepezil hydrochloride (10 mg) Tablet, coated CELTİS İLAÇ SAN. VE TİC. A.Ş. 2020-08-14 Not applicable Turkey SALPEZIL 90/10 MG FILM KAPLI TABLET ,TABLET, 84 ADET Idebenone (90 mg) + Donepezil hydrochloride (10 mg) Tablet, coated CELTİS İLAÇ SAN. VE TİC. A.Ş. 2020-08-14 Not applicable Turkey
Categories
- ATC Codes
- N06BX13 — Idebenone
- Drug Categories
- Chemical TaxonomyProvided by Classyfire
- Description
- This compound belongs to the class of organic compounds known as ubiquinones. These are coenzyme Q derivatives containing a 5, 6-dimethoxy-3-methyl(1,4-benzoquinone) moiety to which an isoprenyl group is attached at ring position 2(or 6).
- Kingdom
- Organic compounds
- Super Class
- Lipids and lipid-like molecules
- Class
- Prenol lipids
- Sub Class
- Quinone and hydroquinone lipids
- Direct Parent
- Ubiquinones
- Alternative Parents
- Fatty alcohols / P-benzoquinones / Vinylogous esters / Primary alcohols / Organic oxides / Hydrocarbon derivatives
- Substituents
- Alcohol / Aliphatic homomonocyclic compound / Carbonyl group / Cyclic ketone / Fatty acyl / Fatty alcohol / Hydrocarbon derivative / Ketone / Organic oxide / Organic oxygen compound
- Molecular Framework
- Aliphatic homomonocyclic compounds
- External Descriptors
- primary alcohol, benzoquinones (CHEBI:31687)
- Affected organisms
- Not Available
Chemical Identifiers
- UNII
- HB6PN45W4J
- CAS number
- 58186-27-9
- InChI Key
- JGPMMRGNQUBGND-UHFFFAOYSA-N
- InChI
- InChI=1S/C19H30O5/c1-14-15(12-10-8-6-4-5-7-9-11-13-20)17(22)19(24-3)18(23-2)16(14)21/h20H,4-13H2,1-3H3
- IUPAC Name
- 2-(10-hydroxydecyl)-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione
- SMILES
- COC1=C(OC)C(=O)C(CCCCCCCCCCO)=C(C)C1=O
References
- General References
- Bodmer M, Vankan P, Dreier M, Kutz KW, Drewe J: Pharmacokinetics and metabolism of idebenone in healthy male subjects. Eur J Clin Pharmacol. 2009 May;65(5):493-501. doi: 10.1007/s00228-008-0596-1. Epub 2009 Jan 6. [Article]
- Zs -Nagy I: Chemistry, toxicology, pharmacology and pharmacokinetics of idebenone: a review. Arch Gerontol Geriatr. 1990 Nov-Dec;11(3):177-86. [Article]
- Gillis JC, Benefield P, McTavish D: Idebenone. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic use in age-related cognitive disorders. Drugs Aging. 1994 Aug;5(2):133-52. [Article]
- EMA Product Label [Link]
- TITCK Product Information: Everon (idebenone/memantine hydrochloride) film-coated tablets [Link]
- External Links
- Human Metabolome Database
- HMDB0253376
- KEGG Drug
- D01750
- PubChem Compound
- 12881464
- PubChem Substance
- 310265009
- ChemSpider
- 3558
- BindingDB
- 50505498
- 51296
- ChEBI
- 31687
- ChEMBL
- CHEMBL252556
- ZINC
- ZINC000001542890
- Drugs.com
- Drugs.com Drug Page
- Wikipedia
- Idebenone
Clinical Trials
- Clinical Trials
Phase Status Purpose Conditions Count 4 Completed Treatment Leber’s hereditary optic neuropathy 1 4 Recruiting Prevention Migraine 1 4 Recruiting Treatment Post stroke epilepsy 1 3 Completed Treatment Ambulatory Care / Duchenne Muscular Dystrophy (DMD) 1 3 Completed Treatment Friedreich's Ataxia 5 3 Recruiting Treatment Headache Disorders / Migraine 1 3 Terminated Treatment Duchenne Muscular Dystrophy (DMD) 2 3 Withdrawn Not Available Leber’s hereditary optic neuropathy 1 2 Completed Treatment Duchenne Muscular Dystrophy (DMD) 2 2 Completed Treatment Friedreich's Ataxia 1
Pharmacoeconomics
- Manufacturers
- Not Available
- Packagers
- Not Available
- Dosage Forms
Form Route Strength Tablet, film coated Oral Tablet Oral 150 mg Tablet, coated Tablet, coated Oral Tablet Oral 30 mg Tablet, film coated Oral 45 mg Tablet, film coated Oral 90 mg Tablet Oral 30.0000 mg Tablet, film coated Oral Tablet, coated 45 MG Tablet, film coated Oral 150 MG Tablet, coated - Prices
- Not Available
- Patents
- Not Available
Properties
- State
- Solid
- Experimental Properties
- Not Available
- Predicted Properties
Property Value Source Water Solubility 0.0163 mg/mL ALOGPS logP 3.98 ALOGPS logP 3.57 Chemaxon logS -4.3 ALOGPS pKa (Strongest Acidic) 16.84 Chemaxon pKa (Strongest Basic) -2 Chemaxon Physiological Charge 0 Chemaxon Hydrogen Acceptor Count 5 Chemaxon Hydrogen Donor Count 1 Chemaxon Polar Surface Area 72.83 Å2 Chemaxon Rotatable Bond Count 12 Chemaxon Refractivity 96.52 m3·mol-1 Chemaxon Polarizability 39.56 Å3 Chemaxon Number of Rings 1 Chemaxon Bioavailability 1 Chemaxon Rule of Five Yes Chemaxon Ghose Filter Yes Chemaxon Veber's Rule No Chemaxon MDDR-like Rule No Chemaxon - Predicted ADMET Features
- Not Available
Spectra
- Mass Spec (NIST)
- Not Available
- Spectra
Spectrum Spectrum Type Splash Key Predicted MS/MS Spectrum - 10V, Positive (Annotated) Predicted LC-MS/MS Not Available Predicted MS/MS Spectrum - 20V, Positive (Annotated) Predicted LC-MS/MS Not Available Predicted MS/MS Spectrum - 40V, Positive (Annotated) Predicted LC-MS/MS Not Available Predicted MS/MS Spectrum - 10V, Negative (Annotated) Predicted LC-MS/MS Not Available Predicted MS/MS Spectrum - 20V, Negative (Annotated) Predicted LC-MS/MS Not Available Predicted MS/MS Spectrum - 40V, Negative (Annotated) Predicted LC-MS/MS Not Available LC-MS/MS Spectrum - LC-ESI-qTof , Positive LC-MS/MS Not Available LC-MS/MS Spectrum - LC-ESI-qTof , Positive LC-MS/MS Not Available MS/MS Spectrum - , positive LC-MS/MS splash10-0072-0944000000-a92c002a2a095f43babd MS/MS Spectrum - , positive LC-MS/MS splash10-0002-0932000000-16596a60138e58f99403
Drug created at August 28, 2015 22:33 / Updated at September 05, 2022 12:50