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Coenzyme Q 10 reverses pathological phenotype and reduces apoptosis in familial CoQ 10 deficiency
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.
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Cited by
Effects of Coenzyme Q10 in Early Parkinson Disease: Evidence of Slowing of the Functional Decline
Shults, C. W. (2002) · Archives of Neurology · doi:10.1001/archneur.59.10.1541 · Cited by 984
Statin Adverse Effects: A Review of the Literature and Evidence for a Mitochondrial Mechanism
Golomb, B. A., & Evans, M. A. (2008) · American Journal of Cardiovascular Drugs · doi:10.2165/0129784-200808060-00004 · Cited by 677
Huynh, K., Bernardo, B. C., McMullen, J. R., & Ritchie, R. H. (2014) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2014.01.003 · Cited by 553
James, A. M., Cochemé, H. M., Smith, R. A. J., & Murphy, M. P. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m501527200 · Cited by 353
Quinzii, C., Naini, A., Salviati, L., Trevisson, E., Navas, P., DiMauro, S., & Hirano, M. (2006) · The American Journal of Human Genetics · doi:10.1086/500092 · Cited by 353
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Metadata
- Authors
- Di Giovanni, S., Mirabella, M., Spinazzola, A., Crociani, P., Silvestri, G., Broccolini, A., Tonali, P., Di Mauro, S., & Servidei, S.
- Venue
- Neurology
- Published
- 2001-08-14
- Volume
- 57
- Issue
- 3
- Pages
- 515-518
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2134855793
- Cited by
- 160
- References
- 0
- FWCI
- 2.71
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Coenzyme Q 10 reverses pathological phenotype and reduces apoptosis in familial CoQ 10 deficiency. https://app.cochat.ai/papers/10.1212/wnl.57.3.515
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- Di Giovanni, S., Mirabella, M., Spinazzola, A., Crociani, P., Silvestri, G., Broccolini, A., Tonali, P., Di Mauro, S., & Servidei, S. (2001). Coenzyme Q 10 reverses pathological phenotype and reduces apoptosis in familial CoQ 10 deficiency. Neurology. https://doi.org/10.1212/wnl.57.3.515
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