Mitochondrial complex III deficiency
MONDO:0015448A rare, genetic, mitochondrial oxidative phosphorylation disorder characterized by a wide spectrum of clinical manifestations ranging from isolated myopathy or transient hepatopathy to severe multisystem disorder (that may include hypotonia, failure to thrive, psychomotor delay, cardiomyopathy, encephalopathy, renal tubulopathy, hearing impairment, lactic acidosis, hypoglycemia and other signs and symptoms).
Also known as: isolated CoQ-cytochrome C reductase deficiency, isolated coenzyme Q-cytochrome C reductase deficiency, isolated mitochondrial respiratory chain complex III deficiency, isolated ubiquinone-cytochrome C reductase deficiency, mitochondrial respiratory chain complex III deficiency, isolated complex III deficiency
13 clinical trials for this condition and its sub-types, 0 tagged with Mitochondrial complex III deficiency itself.
Follow this condition to get notified about new trialsWhere it sits in the disease tree
Browse by category →Sub-types of Mitochondrial complex III deficiency
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11 sub-types
- Mitochondrial complex III deficiency nuclear type 1 0 trials Sub-types →
- Mitochondrial complex III deficiency nuclear type 2 0 trials
- Mitochondrial complex III deficiency nuclear type 3 0 trials
- Mitochondrial complex III deficiency nuclear type 4 0 trials
- Mitochondrial complex III deficiency nuclear type 5 0 trials
- Mitochondrial complex III deficiency nuclear type 6 0 trials
- Mitochondrial complex III deficiency nuclear type 7 0 trials
- Mitochondrial complex III deficiency nuclear type 8 0 trials
- Mitochondrial complex III deficiency nuclear type 9 0 trials
- Mitochondrial complex III deficiency, nuclear type 10 0 trials
- Mitochondrial complex III deficiency, nuclear type 11 0 trials
Most studied deeper sub-types
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New drug shows promise for rare energy disorder
Disease control CompletedThis study tested a new drug called OMT-28 in 28 people with primary mitochondrial disease, a condition that affects how cells produce energy. Participants took the drug once daily for 6 months, and researchers measured safety, blood markers of inflammation, and symptoms like fat…
Phase 2 • Sponsor: Omeicos Therapeutics GmbH • Aim: Disease control
Last updated Jun 27, 2026 08:13 UTC
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Long-Term safety of mitochondrial drug confirmed in 101 patients
Disease control CompletedThis study looked at the safety of vatiquinone in 101 people with inherited mitochondrial disease who had already taken the drug in a previous study or treatment plan. The goal was to track any side effects until the drug became commercially available or the program ended. Partic…
Phase 3 • Sponsor: PTC Therapeutics • Aim: Disease control
Last updated Jun 27, 2026 08:05 UTC