ArticleJournal of inherited metabolic disease2001
Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone.
Article in Journal of inherited metabolic disease, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
10 citing papers in PubMed, 62 citations in OpenAlex.
- Idebenone: Clinical Potential Beyond Neurological Diseases.Drug design, development and therapy · 2025Review
- Mitochondrial Calcium Overload Plays a Causal Role in Oxidative Stress in the Failing Heart.Biomolecules · 2023Review
- Skeletal Muscle Mitochondrial Dysfunction and Oxidative Stress in Peripheral Arterial Disease: A Unifying Mechanism and Therapeutic Target.Antioxidants (Basel, Switzerland) · 2020Review
- Targeting mitochondrial dysfunction and oxidative stress in heart failure: Challenges and opportunities.Free radical biology & medicine · 2018Review
- Expert consensus document: Mitochondrial function as a therapeutic target in heart failure.Nature reviews. Cardiology · 2017Review
- The Value of Coenzyme QJournal of clinical medicine · 2017Review
- Refractory epilepsy and mitochondrial dysfunction due to GM3 synthase deficiency.European journal of human genetics : EJHG · 2013Article
- Mitochondrial energetics and therapeutics.Annual review of pathology · 2010Review
- Protective effects of Fe-Aox29, a novel antioxidant derived from a molecular combination of Idebenone and vitamin E, in immortalized fibroblasts and fibroblasts from patients with Friedreich Ataxia.Molecular and cellular biochemistry · 2007Article
- Clinical presentations of mitochondrial cardiomyopathies.Pediatric cardiologyArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Idebenone, a synthetic analogue of coenzyme Q10, has been shown to improve cardiac function in patients with Friedreich ataxia and a deficiency of respiratory chain complexes I-III. We describe a woman with severe combined right and left heart failure due to a mitochondrial cardiomyopathy. The patient underwent an endomyocardial biopsy as part of an evaluation for cardiac transplantation. It showed severely decreased respiratory complex activities dependent on CoQ, pointing to CoQ depletion. Following idebenone treatment there was a dramatic improvement in her clinical status with resolution of the heart failure.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.