Translating the basic knowledge of mitochondrial functions to metabolic therapy: Role of L-carnitine

Santica M. Marcovina*, Cesare Sirtori, Andrea Peracino, Mihai Gheorghiade, Peggy Borum, Giuseppe Remuzzi, Hossein Ardehali

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

79 Scopus citations

Abstract

Mitochondria play important roles in human physiological processes, and therefore, their dysfunction can lead to a constellation of metabolic and nonmetabolic abnormalities such as a defect in mitochondrial gene expression, imbalance in fuel and energy homeostasis, impairment in oxidative phosphorylation, enhancement of insulin resistance, and abnormalities in fatty acid metabolism. As a consequence, mitochondrial dysfunction contributes to the pathophysiology of insulin resistance, obesity, diabetes, vascular disease, and chronic heart failure. The increased knowledge on mitochondria and their role in cellular metabolism is providing new evidence that these disorders may benefit from mitochondrial-targeted therapies. We review the current knowledge of the contribution of mitochondrial dysfunction to chronic diseases, the outcomes of experimental studies on mitochondrial-targeted therapies, and explore the potential of metabolic modulators in the treatment of selected chronic conditions. As an example of such modulators, we evaluate the efficacy of the administration of L-carnitine and its analogues acetyl and propionyl L-carnitine in several chronic diseases. L-carnitine is intrinsically involved in mitochondrial metabolism and function as it plays a key role in fatty acid oxidation and energy metabolism. In addition to the transportation of free fatty acids across the inner mitochondrial membrane, L-carnitine modulates their oxidation rate and is involved in the regulation of vital cellular functions such as apoptosis. Thus, L-carnitine and its derivatives show promise in the treatment of chronic conditions and diseases associated with mitochondrial dysfunction but further translational studies are needed to fully explore their potential.

Original languageEnglish (US)
Pages (from-to)73-84
Number of pages12
JournalTranslational Research
Volume161
Issue number2
DOIs
StatePublished - Feb 2013

Keywords

  • ATP
  • BP
  • CoA
  • CoASH
  • FA
  • Lp(a)
  • NO
  • OXPHOS
  • ROS
  • adenosine-5'-triphosphate
  • blood pressure
  • coenzyme A
  • eNOS
  • endothelial nitric oxide synthase
  • fatty acid
  • free coenzyme A
  • lipoprotein(a)
  • nitric oxide
  • oxidative-phosphorylation system
  • reactive oxygen species

ASJC Scopus subject areas

  • Public Health, Environmental and Occupational Health
  • Biochemistry, medical
  • Physiology (medical)

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