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Uobiquinine for mitochondrial dises – proCoenzyme Q10 (CoQ10), also known as ubiquinone, is a fat-soluble, vitamin-like molecule naturally present in every cellular membrane within our bodies. This enzyme is a regular component of our diet, although it is also synthesized endogenously. Although CoQ10 lacks approval from the United States Food and Drug Administration (FDA) for treating any medical condition, it is readily accessible as an over-the-counter dietary supplement and is often recommended by both primary care clinicians and specialists.
biquinol is the reduced and more bioavailable form of CoQ10, which plays a central role in mitochondrial biochemistry through its role in shuttling electrons from respiratory chain complexes I or II and the electron transport flavoprotein to respiratory chain complex III. Ubiquinol also serves as a lipophilic and vitamin E-sparing antioxidant, with similar in vitro efficacy

Ubiquinol powder (CoQ10) 145 milligram twice a day si being used to block oxidative damage and redox signaling, and exogenous ubiquinones have been widely used for this purpose in mitochondrial studies. While positive therapeutic effects have been observed with CoQ10 supplementation in humans, its oral bioavailability is poor due to its extreme hydrophobicity (Fig. 1B). Consequently, only a small fraction of orally administered CoQ10 reaches the circulatory system, and augmentation of mitochondrial CoQ10 content is lower still. Therefore, the beneficial effects of exogenous CoQ10 require high doses and long term administration, and only subjects whose CoQ10 levels have been depleted by defective synthesis, age, or disease are responsive.

Perhapd due to the rarity of this condition, the role of ubiquinone is not clear. The doses are high and it should be considered E/I and not medically necessary until definite guideline recommendations or clinical tirals support its effectiveness..

Yuruk Yildirim Z, Toksoy G, Uyguner O, Nayir A, Yavuz S, Altunoglu U, Turkkan ON, Sevinc B, Gokcay G, Kurkcu Gunes D, Kiyak A, Yilmaz A. Primary coenzyme Q10 Deficiency-6 (COQ10D6): Two siblings with variable expressivity of the renal phenotype. Eur J Med Genet. 2020 Jan;63(1):103621.

James AM, Cochemé HM, Smith RA, Murphy MP. Interactions of mitochondria-targeted and untargeted ubiquinones with the mitochondrial respiratory chain and reactive oxygen species. Implications for the use of exogenous ubiquinones as therapies and experimental tools. J Biol Chem. 2005 Jun 3;280(22):21295-312. doi: 10.1074/jbc.M501527200. Epub 2005 Mar 23. PMID: 15788391.

James AM, Cochemé HM, Smith RA, Murphy MP. Interactions of mitochondria-targeted and untargeted ubiquinones with the mitochondrial respiratory chain and reactive oxygen species. Implications for the use of exogenous ubiquinones as therapies and experimental tools. J Biol Chem. 2005 Jun 3;280(22):21295-312. doi: 10.1074/jbc.M501527200. Epub 2005 Mar 23. PMID: 15788391.

Barcelos I, Shadiack E, Ganetzky RD, Falk MJ. Mitochondrial medicine therapies: rationale, evidence, and dosing guidelines. Curr Opin Pediatr. 2020 Dec;32(6):707-718. doi: 10.1097/MOP.0000000000000954. PMID: 33105273; PMCID: PMC7774245.