Global InformationTriheptanoin information
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| Pronunciation | /traɪˈhɛptənɔɪn/ try-HEP-tə-noyn |
| Trade names | Dojolvi |
| Other names | UX007 |
| AHFS/Drugs.com | Professional Drug Facts |
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| Routes of administration | By mouth |
| Drug class | Glycerolipids |
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| ECHA InfoCard | 100.009.681 |
| Chemical and physical data | |
| Formula | C24H44O6 |
| Molar mass | 428.610 g·mol−1 |
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Triheptanoin, sold under the brand name Dojolvi, is a medication for the treatment of children and adults with molecularly confirmed long-chain fatty acid oxidation disorders (LC-FAOD).[2][3][4]
The most common adverse reactions include abdominal pain, diarrhea, vomiting, and nausea.[2][3][4]
Triheptanoin was approved for medical use in the United States in June 2020.[5][3][4]
Triheptanoin is a triglyceride that is composed of three seven-carbon (C7:0) fatty acids. These odd-carbon fatty acids are able to provide anaplerotic substrates for the TCA cycle. Triheptanoin is used clinically in humans to treat inherited metabolic diseases, such as pyruvate carboxylase deficiency and carnitine palmitoyltransferase II deficiency. It also appears to increase the efficacy of the ketogenic diet as a treatment for epilepsy.
Since triheptanoin is composed of odd-carbon fatty acids, it can produce ketone bodies with five carbon atoms, as opposed to even-carbon fatty acids which are metabolized to ketone bodies with four carbon atoms. The five-carbon ketones produced from triheptanoin are beta-ketopentanoate and beta-hydroxypentanoate. Each of these ketone bodies easily crosses the blood–brain barrier and enters the brain.
- ^ "Summary Basis of Decision (SBD) for Dojolvi". Health Canada. 23 October 2014. Retrieved 29 May 2022.
- ^ a b c "Dojolvi- triheptanoin liquid". DailyMed. 30 June 2020. Retrieved 24 September 2020.
- ^ a b c Cite error: The named reference
Ultragenyx PRwas invoked but never defined (see the help page). - ^ a b c "Drug Trials Snapshots: Dojolvi". U.S. Food and Drug Administration. 30 June 2020. Retrieved 16 July 2020.
- ^ Cite error: The named reference
FDA Dojolviwas invoked but never defined (see the help page).