Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

J Child Neurol. 2016 Nov;31(13):1489-1494. doi: 10.1177/0883073816661458. Epub 2016 Aug 3.


There are indications that preexisting mitochondrial disorders or beta-oxidation defects predispose for propofol infusion syndrome. This review aimed at investigating if propofol infusion syndrome occurs exclusively in patients with mitochondrial disorder and if propofol can unmask a mitochondrial disorder. Propofol infusion syndrome has been reported in genetically confirmed mitochondrial disorder patients. In addition, muscle biopsy of patients with propofol infusion syndrome revealed complex IV or complex II deficiency. In animal studies propofol disrupted the electron flow along the respiratory chain and decreased complex I, complex II, and complex III of the respiratory chain. In addition, propofol disrupted the permeability transition pore and reduced the mitochondrial membrane potential. In conclusion, propofol is mitochondrion-toxic and mitochondrial disorder patients should not receive propofol in high dosages over a prolonged period of time. Short-term application of propofol should be safe even in mitochondrial disorder patients. Not only does propofol infusion syndrome occur in mitochondrial disorder patients, but mitochondrial disorder patients are likely at higher risk to develop propofol infusion syndrome. Patients who develop propofol infusion syndrome should be screened for mitochondrial disorder. Propofol infusion syndrome is preventable if risk factors are thoroughly assessed, and if long-term propofol is avoided in patients at risk for propofol infusion syndrome.

Keywords: electron transport; mitochondrial disorder; mitochondrion; myopathy; propofol; respiratory chain; rhabdomyolysis; side effects; toxicity.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Mitochondria / drug effects*
  • Mitochondrial Diseases / diagnosis*
  • Mitochondrial Diseases / epidemiology
  • Mitochondrial Diseases / metabolism*
  • Propofol / toxicity*
  • Risk


  • Propofol