The effects of coenzyme Q 10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation

Suzuki, Susumu; Hinokio, Yoshinori; Ohtomo, Masataka; Hirai, Masashi; Hirai, Atsuko; Chiba, Masaki; Kasuga, Shigeru; Satoh, Yoshinori; Akai, Hiroaki; Toyota, Takayoshi

doi:10.1007/s001250050950

Cited by 107 publications

(62 citation statements)

References 22 publications

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“…This may be due to concerns about toxicity and consequent inadequate dosing or the inability to deliver agents to target sites of ROS production. Attempts to treat diabetes, either in rodents (220,264) or in humans (13,133,220,302), have met with variable results, showing either a beneficial effect on markers of oxidative damage (182,302) or glycemia (220), no effect (13,133), or mixed results depending on tissue and markers of oxidative damage (264). CoQ effectively reduced elevated insulin concentrations, improved endothelial function, and reduced markers of oxidative damage when administered through the drinking water to variants of the spontaneously hypertensive rat (SHR), that have features of the metabolic syndrome (182).…”

Section: B Controlling Ros Production and Oxidative Damagementioning

confidence: 99%

Mitochondrial Dysfunction in Diabetes: From Molecular Mechanisms to Functional Significance and Therapeutic Opportunities

Sivitz

Yorek

2010

Antioxidants & Redox Signaling

636

474

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Given their essential function in aerobic metabolism, mitochondria are intuitively of interest in regard to the pathophysiology of diabetes. Qualitative, quantitative, and functional perturbations in mitochondria have been identified and affect the cause and complications of diabetes. Moreover, as a consequence of fuel oxidation, mitochondria generate considerable reactive oxygen species (ROS). Evidence is accumulating that these radicals per se are important in the pathophysiology of diabetes and its complications. In this review, we first present basic concepts underlying mitochondrial physiology. We then address mitochondrial function and ROS as related to diabetes. We consider different forms of diabetes and address both insulin secretion and insulin sensitivity. We also address the role of mitochondrial uncoupling and coenzyme Q. Finally, we address the potential for targeting mitochondria in the therapy of diabetes. Antioxid. Redox Signal. 12, 537-577.

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Section: B Controlling Ros Production and Oxidative Damagementioning

confidence: 99%

Mitochondrial Dysfunction in Diabetes: From Molecular Mechanisms to Functional Significance and Therapeutic Opportunities

Sivitz

Yorek

2010

Antioxidants & Redox Signaling

636

474

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show abstract

“…The other consequence is the formation of the`apoptosome' in the cytosol, a pro-apoptotic complex comprising cytochrome c, apaf-1, and procaspase-9 idants or specific electron transfer mediators. For example, MIDD is successfully treated with coenzyme Q10, 16 and defects in electron chain components can be bridged with appropriate reductants. 17 Limiting exposure to light may also be useful to control A2E formation and/or its cell toxicity.…”

Section: ± 11mentioning

confidence: 99%

Insight into age-related macular degeneration: New vision in sight

Richter

2001

Cell Death Differ

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“…In addition, Co Q10 has also antioxidant and membrane stabilizing properties. There are previous reports showing benefi cial effects of CoQ10 on some neuromuscular symptoms, prevention of progressive insulin secretory defect, exercise intolerance, hearing loss and myocardial dysfunction in MIDD patients (20)(21)(22)(23).…”

Section: Introductionmentioning

confidence: 97%

Unusual occurrence of intestinal pseudo obstruction in a patient with maternally inherited diabetes and deafness (MIDD) and favorable outcome with coenzyme Q10

Bergamin

Rolim

Dib

et al. 2008

Arq Bras Endocrinol Metab

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Maternally inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. This subtype of diabetes is characterized by maternal transmission, young age at onset and bilateral hearing impairment. Besides diabetes and deafness, the main diagnostic features, a wide range of multisystemic symptoms may be associated with the A3243G mutation. Organs that are most metabolically active, such as muscles, myocardium, retina, cochlea, kidney and brain are frequently affected. Gastrointestinal tract symptoms are also common in patients with mitochondrial disease and constipation and diarrhea are the most frequent manifestations. However, there are few prior reports of intestinal pseudo obstruction in MIDD patients. Here we report the case of a patient with MIDD associated with the mtDNA A3243G mutation who developed chronic intestinal pseudo obstruction, and the introduction of Coenzyme Q10 as adjunctive therapy led to a solution of the pseudo obstruction.

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The effects of coenzyme Q 10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation

Cited by 107 publications

References 22 publications

Mitochondrial Dysfunction in Diabetes: From Molecular Mechanisms to Functional Significance and Therapeutic Opportunities

Mitochondrial Dysfunction in Diabetes: From Molecular Mechanisms to Functional Significance and Therapeutic Opportunities

Insight into age-related macular degeneration: New vision in sight

Unusual occurrence of intestinal pseudo obstruction in a patient with maternally inherited diabetes and deafness (MIDD) and favorable outcome with coenzyme Q10

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