Delivering healthy mitochondria for the therapy of mitochondrial diseases and beyond

Int J Biochem Cell Biol. 2014 Aug:53:141-6. doi: 10.1016/j.biocel.2014.05.009. Epub 2014 May 16.

Abstract

Mitochondrial transfer has been demonstrated to a play a physiological role in the rescuing of mitochondrial DNA deficient cells by co-culture with human mesenchymal stem cells. The successful replacement of mitochondria using microinjection into the embryo has been revealed to improve embryo maturation. Evidence of mitochondrial transfer has been shown to minimize injury of the ischemic-reperfusion rabbit heart model. In this mini review, the therapeutic strategies of mitochondrial diseases based on the concept of mitochondrial transfer are illustrated, as well as a novel approach to peptide-mediated mitochondrial delivery. The possible mechanism of peptide-mediated mitochondrial delivery in the treatment of the myoclonic epilepsy and ragged-red fiber disease is summarized. Understanding the feasibility of mitochondrial manipulation in cells facilitates novel therapeutic skills in the future clinical practice of mitochondrial disorder.

Keywords: Mitochondrial diseases; Mitochondrial transfer; Pep-1; Therapeutics; Transplantation.

Publication types

  • Review

MeSH terms

  • Animals
  • Cysteamine / analogs & derivatives*
  • Cysteamine / therapeutic use
  • DNA, Mitochondrial / genetics
  • Humans
  • Mitochondria / pathology*
  • Mitochondria / transplantation
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / pathology
  • Mitochondrial Diseases / therapy*
  • Peptides / therapeutic use*
  • Rabbits

Substances

  • DNA, Mitochondrial
  • Pep-1 peptide
  • Peptides
  • Cysteamine