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Related Experiment Video

Updated: Jun 9, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess

Published on: June 1, 2022

Coenzyme Q and mitochondrial disease.

Catarina M Quinzii¹, Michio Hirano

¹Department of Neurology, Columbia University Medical Center, 630 West 168th Street, New York, NY 10032, USA.

Developmental Disabilities Research Reviews

|September 7, 2010

Summary

Coenzyme Q(10) deficiency is a heterogeneous syndrome affecting multiple systems, often inherited recessively. Supplementation with Coenzyme Q(10) can be beneficial for patients with this condition.

Area of Science:

Biochemistry
Genetics
Mitochondrial Biology

Background:

Coenzyme Q(10) (CoQ(10)) is vital for mitochondrial respiration and acts as an antioxidant.
CoQ(10) deficiency presents heterogeneously, with autosomal recessive inheritance and responsiveness to CoQ(10) therapy.
Clinical phenotypes include encephalomyopathy, severe infantile multisystemic disease, cerebellar ataxia, isolated myopathy, and nephrotic syndrome.

Purpose of the Study:

To review the clinical and molecular aspects of Coenzyme Q(10) deficiency.
To discuss the genetic basis of primary and secondary CoQ(10) deficiencies.
To highlight the pathogenesis and potential therapeutic strategies for CoQ(10) deficiency.

Main Methods:

Literature review of studies on Coenzyme Q(10) deficiency.

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Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

Published on: November 23, 2011

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Last Updated: Jun 9, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess

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Published on: June 1, 2022

Inner Mitochondrial Membrane Sensitivity to Na+ Reveals Partially Segmented Functional CoQ Pools

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Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

Published on: November 23, 2011

Analysis of genetic mutations associated with CoQ(10) biosynthesis.

Examination of pathogenetic mechanisms including respiratory chain defects and oxidative stress.

Main Results:

Identified five major clinical phenotypes associated with CoQ(10) deficiency.
Distinguished between primary CoQ(10) deficiencies (biosynthesis gene mutations) and secondary deficiencies.
Linked pathogenesis to respiratory chain dysfunction, reactive oxygen species (ROS) production, and apoptosis.

Conclusions:

Coenzyme Q(10) deficiency is a complex disorder with diverse clinical presentations.
Understanding genetic defects is crucial for diagnosis and management.
Further in vitro and in vivo research is needed to refine therapeutic approaches for CoQ(10) deficiency.