Drugs interfering with mitochondrial disorders

Drug Chem Toxicol. 2010 Apr;33(2):138-51. doi: 10.3109/01480540903207076.


Objectives: Treatment of mitochondrial disorders (MIDs) is a challenge, since there is no causal therapy available and since there are few studies available, which demonstrate an effect of any agents offered for symptomatic or supportive therapy. Treatment of MIDs is based on five main columns, including symptomatic measures, application of supportive agents, dietary measures, physiotherapy, and the avoidance of drugs known to be toxic for mitochondrial functions. This review aims to give an overview about those agents that interfere with mitochondrial functions.

Results: Mitochondrion-toxic agents include corticosteroids, valproic acid, phenytoin, barbiturates, propofol, volatile anesthetics, nondepolarizing muscle relaxants, some local anesthetics, statins, fibrates, biguanides, glitazones, beta-blockers, amiodarone, some neuroleptics, some antibiotics, some chemotherapeutics, nucleoside reverse transcriptase inhibitors, and various other drugs. These agents should be avoided or given only under close monitoring, although some of them also exhibit beneficial effects, possibly due to the peculiarities of mitochondrial genetics. Typical side effects may be steroid myopathy, propofol syndrome, statin/fibrate myopathy, neuroleptic-induced extrapyramidal signs, or zidovudine myopathy.

Conclusions: Avoiding or discontinuing mitochondrion-toxic drugs in MID patients can have a significant impact on the course and outcome of these patients.

Publication types

  • Review

MeSH terms

  • Animals
  • Contraindications
  • Drug-Related Side Effects and Adverse Reactions*
  • Humans
  • Mitochondria / drug effects*
  • Mitochondria / genetics
  • Mitochondria / metabolism
  • Mitochondrial Diseases / diagnosis
  • Mitochondrial Diseases / drug therapy*
  • Pharmaceutical Preparations


  • Pharmaceutical Preparations