Peptide-mediated delivery of donor mitochondria improves mitochondrial function and cell viability in human cybrid cells with the MELAS A3243G mutation

Sci Rep. 2017 Sep 6;7(1):10710. doi: 10.1038/s41598-017-10870-5.

Abstract

The cell penetrating peptide, Pep-1, has been shown to facilitate cellular uptake of foreign mitochondria but further research is required to evaluate the use of Pep-1-mediated mitochondrial delivery (PMD) in treating mitochondrial defects. Presently, we sought to determine whether mitochondrial transplantation rescue mitochondrial function in a cybrid cell model of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) disease. Following PMD, recipient cells had internalized donor mitochondria after 1 h, and expressed higher levels of normal mitochondrial DNA, particularly at the end of the treatment and 11 days later. After 4 days, mitochondrial respiratory function had recovered and biogenesis was evident in the Pep-1 and PMD groups, compared to the untreated MELAS group. However, only PMD was able to reverse the fusion-to-fission ratio of mitochondrial morphology, and mitochondria shaping proteins resembled the normal pattern seen in the control group. Cell survival following hydrogen peroxide-induced oxidative stress was also improved in the PMD group. Finally, we observed that PMD partially normalized cytokine expression, including that of interleukin (IL)-7, granulocyte macrophage-colony-stimulating factor (GM-CSF), and vascular endothelial growth factor (VEGF), in the MELAS cells. Presently, our data further confirm the protective effects of PMD as well in MELAS disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autophagy
  • Cell Line, Tumor
  • Cell Respiration
  • Cell Survival
  • Cell-Penetrating Peptides / metabolism*
  • Cytokines / biosynthesis
  • Genotyping Techniques
  • Humans
  • MELAS Syndrome / genetics*
  • MELAS Syndrome / metabolism*
  • MELAS Syndrome / therapy
  • Mitochondria / genetics*
  • Mitochondria / metabolism*
  • Mitochondria / transplantation
  • Mitochondria / ultrastructure
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism
  • Mutation*
  • Oxidative Stress
  • Oxygen / metabolism
  • Staining and Labeling

Substances

  • Cell-Penetrating Peptides
  • Cytokines
  • Mitochondrial Proteins
  • Oxygen