Related Experiment Video
Updated: Oct 5, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess
Published on: June 1, 2022
Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency
S Di Giovanni1, M Mirabella, A Spinazzola
1Institute of Neurology, Catholic University, Rome, Italy.
Abstract:
Two brothers with myopathic coenzyme Q10 (CoQ10) deficiency responded dramatically to CoQ10 supplementation. Muscle biopsies before therapy showed ragged-red fibers, lipid storage, and complex I + III and II + III deficiency. Approximately 30% of myofibers had multiple features of apoptosis. After 8 months of treatment, excessive lipid storage resolved, CoQ10 level normalized, mitochondrial enzymes increased, and proportion of fibers with TUNEL-positive nuclei decreased to 10%. The authors conclude that muscle CoQ10 deficiency can be corrected by supplementation of CoQ10, which appears to stimulate mitochondrial proliferation and to prevent apoptosis.
Related Concept Videos
Apoptosis
Cellular Injury V: Apoptosis and Autophagy
The Intrinsic Apoptotic Pathway
Mitochondrial Precursor Proteins
Most of the mitochondrial precursors...
Role of Reduced Coenzymes NADH and FADH₂
Electron Transport Chain: Complex I and II
ROS generation is regulated and maintained at moderate levels necessary...
