CoQ10 deficiency diseases in adults

Quinzii, Catarina M.; Hirano, Michio; DiMauro, Salvatore

doi:10.1016/j.mito.2007.03.004

Cited by 60 publications

(39 citation statements)

References 35 publications

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“…Although these strategies have limited positive therapeutic effects, they do not allow for complete recovery of lost function [57][58][59][60]. Without a drug carrier, the tissue distribution, cellular uptake and intracellular trafficking of therapeutic agents into mitochondria cannot be actively controlled and, therefore, are insufficient for therapeutic activity.…”

Section: Mitochondria and Reactive Oxygen Speciesmentioning

confidence: 99%

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Yamada

Harashima

2008

Advanced Drug Delivery Reviews

225

148

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Key words: Mitochondria, mitochondrial drug delivery, mitochondrial macromolecule delivery, MITO-Porter, membrane fusion, multifunctional envelope-type nano device (MEND), mitochondrial protein therapy, mitochondrial gene therapy, mitochondrial protein import machinery, mitochondrial DNA. Yamada, Y., et al. AbstractMitochondrial dysfunction has been implicated in a variety of human disorders-the so-called mitochondrial diseases. Therefore, the organelle is a promising therapeutic drug target. In this review, we describe the key role of mitochondria in living cells, a number of mitochondrial drug delivery systems and mitochondria-targeted therapeutic strategies. In particular, we discuss mitochondrial delivery of macromolecules, such as proteins and nucleic acids. The discussion of protein delivery is limited primarily to the mitochondrial import machinery. In the section on mitochondrial gene delivery and therapy, we discuss mitochondrial diseases caused by mutations in mitochondrial DNA, several gene delivery strategies and approaches to mitochondrial gene therapy. This review also summarizes our current efforts regarding liposome-based delivery system including use of a multifunctional envelope-type nano device (MEND) and mitochondrial liposome-based delivery as anti-cancer therapies. Furthermore, we introduce the novel MITO-Porter-a liposome-based mitochondrial delivery system that functions using a membrane-fusion mechanism.Yamada, Y., et al. 3

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Section: Mitochondria and Reactive Oxygen Speciesmentioning

confidence: 99%

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Yamada

Harashima

2008

Advanced Drug Delivery Reviews

225

148

View full text Add to dashboard Cite

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“…Various authors recommended daily intake of CoQ10 about 30-100 mg for healthy people over 40 years and 60-1200 mg for those undergoing an adjunctive therapy in some medical conditions (Jones et al, 2002, Bonakdar and Guarneri, 2005, Challem, 2005. The CoQ10 level in human tissues vary with inappropriate nutrition, smoking and different medical conditions cardiomyopathy, diabetes and neurological disorder conditions (Elsayed and Bendich, 2001;Quinzii et al, 2007;Fotino et al, 2013;Madmani et al, 2014).…”

Section: Coenzyme Q10 Absorption Among Body Tissuesmentioning

confidence: 99%

Dietary Essentiality I: Coenzyme Q10 Conditionally Essential-Review

Gopi¹,

Kumar²,

Elaiyaraja³

et al. 2015

Asian J. of Animal and Veterinary Advances

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The essentiality of nutrients keeps on changing with the advancement in nutritional research and genetic gain. The genetic gain especially in poultry sector is very high which results in increase in nutrient requirement of both the essential and non-essential nutrients. For the rapid growth the requirement of essential nutrients reaches many folds which are in direct relation with the performance, but the requirement for non-essential nutrients is an indirect one. Most of the dispensable amino acids, vitamin C, carnitine, etc. which are being synthesized endogenously are now a days unable to meet the birds requirements that warrants the dietary supply. Another important nutrient is coenzyme Q10 (CoQ10) which endogenously synthesized is now gaining much attention as a supplement for fast growing broilers. The CoQ10 can be termed as multi-functionary as each and every cell in the body needs this but quantity is being high for very active organs like heart, lungs, liver, kidney, etc. They are essential for cellular oxidative phosphorylation and regenerative antioxidant. The supplementation of CoQ10 improved the feed efficiency with reducing the electron leaks from mitochondria and increases total antioxidant capacity. For this property, CoQ10 is widely used in human medicine especially persons suffering from cardiac, neurological disorder, hypercholesterolemic condition and also even in cancer. The CoQ10 in poultry draws first attention when it is found to reducing the ascites mortality in marketable broilers. Thereafter, the advantages of CoQ10 is started to exploit with much attention to ascites, feed efficiency, cholesterol lowering effect nutraceutical and nutrigenomic property both in poultry and swine industry.

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“…Genetic or acquired defects in its synthesis or metabolism, and interactions with medications such as beta-blockers, hydrochlorothiazide, methyldopa, statin and tricyclic antidepressants may also reduce levels of CoQ 10 (Quinzii, Hirano, DiMauro, 2007b). …”

Section: Introductionmentioning

confidence: 99%

Coenzyme Q10 and its effects in the treatment of neurodegenerative diseases

Santos¹,

Antunes

Santos

et al. 2009

Braz. J. Pharm. Sci.

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According to clinical and pre-clinical studies, oxidative stress and its consequences may be the cause or, at least, a contributing factor, to a large number of neurodegenerative diseases. These diseases include common and debilitating disorders, characterized by progressive and irreversible loss of neurons in specific regions of the brain. The most common neurodegenerative diseases are Parkinson's disease, Huntington's disease, Alzheimer's disease and amyotrophic lateral sclerosis. Coenzyme Q10 (CoQ10) has been extensively studied since its discovery in 1957. It is a component of the electron transportation chain and participates in aerobic cellular respiration, generating energy in the form of adenosine triphosphate (ATP). The property of CoQ10 to act as an antioxidant or a pro-oxidant, suggests that it also plays an important role in the modulation of redox cellular status under physiological and pathological conditions, also performing a role in the ageing process. In several animal models of neurodegenerative diseases, CoQ10 has shown beneficial effects in reducing disease progression. However, further studies are needed to assess the outcome and effectiveness of CoQ10 before exposing patients to unnecessary health risks at significant costs.
De acordo com estudos clínicos e pré-clínicos, o estresse oxidativo e suas conseqüências podem ser a causa, ou, no mínimo, o fator que contribui para grande número de doenças degenerativas. Estas doenças incluem problemas comuns e debilitantes, caracterizados por perda progressiva e irreversível de neurônios em regiões específicas do cérebro. As doenças degenerativas mais comuns são doença de Parkinson, de Hutington, de Alzheimer e esclerose amiotrófica lateral. A Coenzima Q10 (CoQ10) tem sido intensamente estudada desde sua descoberta, em 1957. É um componente da cadeia de transporte eletrônico e participa da respiração aeróbica celular, gerando energia na forma de trifosfato de adenosina (ATP). A propriedade da CoQ10 de atuar como antioxidante ou pró-oxidante sugere papel importante na modulação do estado redox celular sob condições fisiológicas e patológicas, desempenhando, também, papel no processo de envelhecimento. Em vários modelos animais de doenças neurodegenerativas, a CoQ10 mostrou efeitos benéficos na redução do curso da doença. Entretanto, há necessidade de estudos adicionais para avaliar o efeito e a eficácia da CoQ10 antes de expor os pacientes a riscos de saúde desnecessários e de alto custo

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CoQ10 deficiency diseases in adults

Abstract: Deficiency of Coenzyme Q 10 (CoQ 10

Cited by 60 publications

References 35 publications

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Mitochondrial drug delivery systems for macromolecule and their therapeutic application to mitochondrial diseases

Dietary Essentiality I: Coenzyme Q10 Conditionally Essential-Review

Coenzyme Q10 and its effects in the treatment of neurodegenerative diseases

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