MedKoo Cat#: 319685 | Name: Vatiquinone
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Description:

WARNING: This product is for research use only, not for human or veterinary use.

Vatiquinone, also known as EPI 743, is an orally bioavailable para-benzoquinone being developed for inherited mitochondrial diseases. The mechanism of action of EPI-743 involves augmenting the synthesis of glutathione, optimizing metabolic control, enhancing the expression of genetic elements critical for cellular management of oxidative stress, and acting at the mitochondria to regulate electron transport. This product is supplied as a solution: 10mg/mL in ethanol. Bulk quantity can be supplied in pure liquid form.

Chemical Structure

Vatiquinone
Vatiquinone
CAS#1213269-98-7

Theoretical Analysis

MedKoo Cat#: 319685

Name: Vatiquinone

CAS#: 1213269-98-7

Chemical Formula: C29H44O3

Exact Mass: 440.3290

Molecular Weight: 440.67

Elemental Analysis: C, 79.04; H, 10.06; O, 10.89

Price and Availability

Size Price Availability Quantity
5mg USD 150.00 Ready to ship
10mg USD 250.00 Ready to ship
25mg USD 550.00 Ready to ship
50mg USD 950.00 Ready to ship
100mg USD 1,650.00 Ready to ship
200mg USD 2,950.00 Ready to ship
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Related CAS #
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Synonym
EPI743; EPI-743; EPI 743; Vatiquinone; alpha-Tocotrienol quinone. ATQ3;
IUPAC/Chemical Name
2-((R,6E,10E)-3-hydroxy-3,7,11,15-tetramethylhexadeca-6,10,14-trien-1-yl)-3,5,6-trimethylcyclohexa-2,5-diene-1,4-dione
InChi Key
LNOVHERIIMJMDG-XZXLULOTSA-N
InChi Code
InChI=1S/C29H44O3/c1-20(2)12-9-13-21(3)14-10-15-22(4)16-11-18-29(8,32)19-17-26-25(7)27(30)23(5)24(6)28(26)31/h12,14,16,32H,9-11,13,15,17-19H2,1-8H3/b21-14+,22-16+/t29-/m1/s1
SMILES Code
O=C1C(CC[C@](C)(O)CC/C=C(C)/CC/C=C(C)/CC/C=C(C)\C)=C(C)C(C(C)=C1C)=O
Appearance
Oily liquid (sizes under 1g will be supplied in ethanol at 10mg/mL)
Purity
>98% (or refer to the Certificate of Analysis)
Shipping Condition
Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.
Storage Condition
Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).
Solubility
Soluble in DMSO, not in water
Shelf Life
>2 years if stored properly
Drug Formulation
This drug may be formulated in DMSO
Stock Solution Storage
0 - 4 C for short term (days to weeks), or -20 C for long term (months).
HS Tariff Code
2934.99.9001
More Info
Edison Pharmaceuticals is developing vatiquinone, which was awarded Fast Track status for Friedreich's ataxia in March 2014.
Biological target:
Mitochondria
In vitro activity:
Primary fibroblasts and B-lymphocytes derived from patients with mitochondrial disease-associated epilepsy were cultured under standardized conditions. Ferroptosis was induced by treatment with the irreversible GPX4 inhibitor RSL3 or a combination of pharmacological glutathione depletion and excess iron. EPI-743 was co-administered and endpoints, including cell viability and 15-LO-dependent lipid oxidation, were measured. EPI-743 potently prevented ferroptosis in patient cells representing five distinct pediatric disease syndromes with associated epilepsy. Cytoprotection was preceded by a dose-dependent decrease in general lipid oxidation and the specific 15-LO product 15-hydroxyeicosatetraenoic acid (15-HETE). These findings support the continued clinical evaluation of EPI-743 as a therapeutic agent for PCH6 and other mitochondrial diseases with associated epilepsy. Reference: Kahn-Kirby AH, Amagata A, Maeder CI, Mei JJ, Sideris S, Kosaka Y, Hinman A, Malone SA, Bruegger JJ, Wang L, Kim V, Shrader WD, Hoff KG, Latham JC, Ashley EA, Wheeler MT, Bertini E, Carrozzo R, Martinelli D, Dionisi-Vici C, Chapman KA, Enns GM, Gahl W, Wolfe L, Saneto RP, Johnson SC, Trimmer JK, Klein MB, Holst CR. Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsy. PLoS One. 2019 Mar 28;14(3):e0214250. doi: 10.1371/journal.pone.0214250. PMID: 30921410; PMCID: PMC6438538.
In vivo activity:
TBD
Solvent mg/mL mM
Solubility
Ethanol 10.0 22.69
Note: There can be variations in solubility for the same chemical from different vendors or different batches from the same vendor. The following factors can affect the solubility of the same chemical: solvent used for crystallization, residual solvent content, polymorphism, salt versus free form, degree of hydration, solvent temperature. Please use the solubility data as a reference only. Warming and sonication will facilitate dissolving. Still have questions? Please contact our Technical Support scientists.

Preparing Stock Solutions

The following data is based on the product molecular weight 440.67 Batch specific molecular weights may vary from batch to batch due to the degree of hydration, which will affect the solvent volumes required to prepare stock solutions.

Recalculate based on batch purity %
Concentration / Solvent Volume / Mass 1 mg 5 mg 10 mg
1 mM 1.15 mL 5.76 mL 11.51 mL
5 mM 0.23 mL 1.15 mL 2.3 mL
10 mM 0.12 mL 0.58 mL 1.15 mL
50 mM 0.02 mL 0.12 mL 0.23 mL
Formulation protocol:
Kahn-Kirby AH, Amagata A, Maeder CI, Mei JJ, Sideris S, Kosaka Y, Hinman A, Malone SA, Bruegger JJ, Wang L, Kim V, Shrader WD, Hoff KG, Latham JC, Ashley EA, Wheeler MT, Bertini E, Carrozzo R, Martinelli D, Dionisi-Vici C, Chapman KA, Enns GM, Gahl W, Wolfe L, Saneto RP, Johnson SC, Trimmer JK, Klein MB, Holst CR. Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsy. PLoS One. 2019 Mar 28;14(3):e0214250. doi: 10.1371/journal.pone.0214250. PMID: 30921410; PMCID: PMC6438538.
In vitro protocol:
Kahn-Kirby AH, Amagata A, Maeder CI, Mei JJ, Sideris S, Kosaka Y, Hinman A, Malone SA, Bruegger JJ, Wang L, Kim V, Shrader WD, Hoff KG, Latham JC, Ashley EA, Wheeler MT, Bertini E, Carrozzo R, Martinelli D, Dionisi-Vici C, Chapman KA, Enns GM, Gahl W, Wolfe L, Saneto RP, Johnson SC, Trimmer JK, Klein MB, Holst CR. Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsy. PLoS One. 2019 Mar 28;14(3):e0214250. doi: 10.1371/journal.pone.0214250. PMID: 30921410; PMCID: PMC6438538.
In vivo protocol:
TBD
1: Lee L, Flach S, Xue H, Arivelu L, Golden L, Kong R, Darpo B. Lack of Concentration-QTc Relationship and Cardiac Risk With Vatiquinone Therapeutic and Supratherapeutic Doses. Clin Pharmacol Drug Dev. 2024 Nov;13(11):1227-1238. doi: 10.1002/cpdd.1476. Epub 2024 Oct 17. PMID: 39415654. 2: Lee L, Okudaira N, Murase K, Kong R, Jones HM. Determination of Vatiquinone Drug-Drug Interactions, as CYP450 Perpetrator and Victim, Using Physiologically Based Pharmacokinetic (PBPK) Modeling and Simulation. J Clin Pharmacol. 2024 Sep 23. doi: 10.1002/jcph.6133. Epub ahead of print. PMID: 39308341. 3: Lee L, Thoolen M, Ma J, Kaushik D, Golden L, Kong R. Effect of Itraconazole, a CYP3A4 Inhibitor, and Rifampin, a CYP3A4 Inducer, on the Pharmacokinetics of Vatiquinone. Clin Pharmacol Drug Dev. 2024 Dec;13(12):1283-1290. doi: 10.1002/cpdd.1461. Epub 2024 Aug 12. PMID: 39133029; PMCID: PMC11609057. 4: Chang KH, Chen CM. The Role of NRF2 in Trinucleotide Repeat Expansion Disorders. Antioxidants (Basel). 2024 May 26;13(6):649. doi: 10.3390/antiox13060649. PMID: 38929088; PMCID: PMC11200942. 5: Kayser EB, Chen Y, Mulholland M, Truong V, James K, Hanaford A, Johnson S. Evaluating the efficacy of vatiquinone in preclinical models of mitochondrial disease. Res Sq [Preprint]. 2024 Jun 3:rs.3.rs-4202689. doi: 10.21203/rs.3.rs-4202689/v1. PMID: 38883711; PMCID: PMC11177993. 6: Ma J, Lee L, Yao B, Giannousis P, Thoolen M, Ye Q, Golden L, Klein M, Kong R. Absorption, distribution, metabolism and excretion of 14C-vatiquinone in rats, dogs, and human subjects. Xenobiotica. 2023 May;53(5):396-411. doi: 10.1080/00498254.2023.2245459. Epub 2023 Aug 18. PMID: 37552765. 7: Cores Á, Carmona-Zafra N, Clerigué J, Villacampa M, Menéndez JC. Quinones as Neuroprotective Agents. Antioxidants (Basel). 2023 Jul 20;12(7):1464. doi: 10.3390/antiox12071464. PMID: 37508002; PMCID: PMC10376830. 8: Jain P, Badgujar L, Spoorendonk J, Buesch K. Clinical evidence of interventions assessed in Friedreich ataxia: a systematic review. Ther Adv Rare Dis. 2022 Nov 29;3:26330040221139872. doi: 10.1177/26330040221139872. PMID: 37180421; PMCID: PMC10032438. 9: Lee L, Murase K, Ma J, Thoolen M. Clinical Drug-Drug Interaction Between Vatiquinone, a 15-Lipoxygenase Inhibitor, and Rosuvastatin, a Breast Cancer Resistance Protein Substrate. Clin Pharmacol Drug Dev. 2023 Mar;12(3):279-286. doi: 10.1002/cpdd.1199. Epub 2022 Dec 7. PMID: 36478438. 10: Murase K, Lee L, Ma J, Barrett R, Thoolen M. Evaluation of vatiquinone drug- drug interaction potential in vitro and in a phase 1 clinical study with tolbutamide, a CYP2C9 substrate, and omeprazole, a CYP2C19 substrate, in healthy subjects. Eur J Clin Pharmacol. 2022 Nov;78(11):1823-1831. doi: 10.1007/s00228-022-03393-0. Epub 2022 Sep 27. PMID: 36166059. 11: Khan H, Kaur Grewal A, Gurjeet Singh T. Mitochondrial dynamics related neurovascular approaches in cerebral ischemic injury. Mitochondrion. 2022 Sep;66:54-66. doi: 10.1016/j.mito.2022.08.001. Epub 2022 Aug 5. PMID: 35940452. 12: Kahn-Kirby AH, Amagata A, Maeder CI, Mei JJ, Sideris S, Kosaka Y, Hinman A, Malone SA, Bruegger JJ, Wang L, Kim V, Shrader WD, Hoff KG, Latham JC, Ashley EA, Wheeler MT, Bertini E, Carrozzo R, Martinelli D, Dionisi-Vici C, Chapman KA, Enns GM, Gahl W, Wolfe L, Saneto RP, Johnson SC, Trimmer JK, Klein MB, Holst CR. Targeting ferroptosis: A novel therapeutic strategy for the treatment of mitochondrial disease-related epilepsy. PLoS One. 2019 Mar 28;14(3):e0214250. doi: 10.1371/journal.pone.0214250. PMID: 30921410; PMCID: PMC6438538. 13: Finsterer J, Zarrouk-Mahjoub S. Is vatiquinone truly beneficial for Leigh syndrome? Brain Dev. 2018 May;40(5):443. doi: 10.1016/j.braindev.2017.10.002. Epub 2017 Oct 18. PMID: 29054334.