Transport via Niemann-Pick C1 Like 1 contributes to the intestinal absorption of ubiquinone

Nashimoto, Shunsuke; Takekawa, Yuto; Takekuma, Yoh; Sugawara, Mitsuru; Sato, Yuki

doi:10.1016/j.dmpk.2020.08.002

Cited by 13 publications

(6 citation statements)

References 34 publications

(50 reference statements)

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“…This information leads us to suspect the existence of a transporter in the enterocyte wall. So far, only cholesterol Niemann-Pick C1 like transporter is linked to CoQ10 absorption [ 52 ]. CoQ10 exits the enterocytes within the chylomicrons in the process of exocytosis and then is transported with chyle within lymphatic vessels to reach the systemic circulation.…”

Section: Primary Coenzyme Q10 Deficiency-4mentioning

confidence: 99%

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency

et al. 2022

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COQ8A-ataxia is a mitochondrial disease in which a defect in coenzyme Q10 synthesis leads to dysfunction of the respiratory chain. The disease is usually present as childhood-onset progressive ataxia with developmental regression and cerebellar atrophy. However, due to variable phenotype, it may be hard to distinguish from other mitochondrial diseases and a wide spectrum of childhood-onset cerebellar ataxia. COQ8A-ataxia is a potentially treatable condition with the supplementation of coenzyme Q10 as a main therapy; however, even 50% may not respond to the treatment. In this study we review the clinical manifestation and management of COQ8A-ataxia, focusing on current knowledge of coenzyme Q10 supplementation and approach to further therapies. Moreover, the case of a 22-month-old girl with cerebellar ataxia and developmental regression will be presented.

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Section: Primary Coenzyme Q10 Deficiency-4mentioning

confidence: 99%

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency

et al. 2022

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“…The uptake of carotenoids by the enterocytes has been thought to occur by simple diffusion for a long period until the scavenger receptor class B was found to be essential for xanthophyll cellular distribution (Reboul, 2019a). Then, SR‐BI has been shown to be a transporter for lutein, phytoene, phytofluene, α‐carotene, β‐carotene, and β‐cryptoxanthin absorption (Mapelli‐Brahm et al., 2018; Reboul, 2019a); CD36 has been shown to facilitate the enterocyte uptake of lycopene, lutein, α‐carotene, β‐carotene, and β‐cryptoxanthin (Borel et al., 2013; Moussa et al., 2011); NPC1L1 has been shown to be involved in the absorption of α‐carotene, β‐carotene, β‐cryptoxanthin, lutein, and coenzyme Q10 (Nashimoto et al., 2020; Reboul, 2019a). However, the mechanisms involved in absorption of polyphenolic compounds are still not clearly uncovered due to their complicated metabolism.…”

Section: Gastrointestinal Fate Of Nutraceutical Emulsions After Oral ...mentioning

confidence: 99%

A review of recent progress in improving the bioavailability of nutraceutical‐loaded emulsions after oral intake

Chen

Liu

et al. 2022

Comp Rev Food Sci Food Safe

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Increasing awareness of the health benefits of specific constituents in fruits, vegetables, cereals, and other whole foods has sparked a broader interest in the potential health benefits of nutraceuticals. Many nutraceuticals are hydrophobic substances, which means they must be encapsulated in colloidal delivery systems. Oil‐in‐water emulsions are one of the most widely used delivery systems for improving the bioavailability and bioactivity of these nutraceuticals. The composition and structure of emulsions can be designed to improve the water dispersibility, physicochemical stability, and bioavailability of the encapsulated nutraceuticals. The nature of the emulsion used influences the interfacial area and properties of the nutraceutical‐loaded oil droplets in the gastrointestinal tract, which influences their digestion, as well as the bioaccessibility, metabolism, and absorption of the nutraceuticals. In this article, we review recent in vitro and in vivo studies on the utilization of emulsions to improve the bioavailability of nutraceuticals. The findings from this review should facilitate the design of more efficacious nutraceutical‐loaded emulsions with increased bioactivity.

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“…Fat-solubilized formulations are preferred to powder-based presentations as solubilization in the fat matrix permits superior bioavailability [ 178 , 179 ]. Recently, it has been found that CoQ transport in intestinal epithelium requires Niemann-Pick Like 1 protein, a transporter involved in the intestinal absorption of fat-soluble components such as cholesterol [ 180 ]. This is an interesting aspect as people showing low fat absorption from their diet may suffer from an insufficient incorporation of dietary CoQ.…”

Section: Supplementation With Coq: Metabolic Recoverymentioning

confidence: 99%

Coenzyme Q at the Hinge of Health and Metabolic Diseases

et al. 2021

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Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at transcription and translation, but also by environment and energy requirements. Here, we review how coenzyme Q reacts within mitochondria to promote ATP synthesis and also integrates a plethora of metabolic pathways and regulates mitochondrial oxidative stress. Coenzyme Q is also located in all cellular membranes and plasma lipoproteins in which it exerts antioxidant function, and its reaction with different extramitochondrial oxidoreductases contributes to regulate the cellular redox homeostasis and cytosolic oxidative stress, providing a key factor in controlling various apoptosis mechanisms. Coenzyme Q levels can be decreased in humans by defects in the biosynthesis pathway or by mitochondrial or cytosolic dysfunctions, leading to a highly heterogeneous group of mitochondrial diseases included in the coenzyme Q deficiency syndrome. We also review the importance of coenzyme Q levels and its reactions involved in aging and age-associated metabolic disorders, and how the strategy of its supplementation has had benefits for combating these diseases and for physical performance in aging.

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Transport via Niemann-Pick C1 Like 1 contributes to the intestinal absorption of ubiquinone

Cited by 13 publications

References 34 publications

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency

COQ8A-Ataxia as a Manifestation of Primary Coenzyme Q Deficiency

A review of recent progress in improving the bioavailability of nutraceutical‐loaded emulsions after oral intake

Coenzyme Q at the Hinge of Health and Metabolic Diseases

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