Volume 19 Issue 2, February 2018

Volume 19 Issue 2

'Amazing mitochondria' by Kirsten Lee, inspired by this focus issue.

Comment

  • Comment |

    Mary Herbert and Doug Turnbull discuss how the transmission of pathogenic mutations in mitochondrial DNA may be prevented by reproductive techniques designed to replace the mitochondria in eggs from affected women.

    • Mary Herbert
    •  & Doug Turnbull

    Focus:

Research Highlights

Reviews

  • Review Article |

    Mitochondrial disorders encompass a broad range of pathologies, which manifest in different tissues, with variable age of onset and symptoms. Recent findings suggest that mitochondrial stress responses, which are activated by defects in mitochondrial genome maintenance and expression, contribute to cell and systemic dysfunction, and could explain the phenotypic variability of mitochondrial disorders.

    • Anu Suomalainen
    •  & Brendan J. Battersby

    Focus:

  • Review Article |

    Recent studies that combine cell biology, structural and proteomic approaches have unravelled how ubiquitin is conjugated to damaged mitochondria through the PINK1–parkin pathway to promote mitophagy. The findings have revealed links between PINK1–parkin, antigen presentation and neuronal survival and have implications for the understanding of neurological disorders.

    • J. Wade Harper
    • , Alban Ordureau
    •  & Jin-Mi Heo
  • Review Article |

    Cells activate a transcriptional response known as the mitochondrial unfolded protein response (UPRmt) when mitochondrial integrity and function are impaired to promote their recovery. Recent insights into the regulation, mechanisms and functions of the UPRmt have uncovered important links to ageing and ageing-associated diseases.

    • Tomer Shpilka
    •  & Cole M. Haynes
  • Review Article |

    AMP-activated protein kinase (AMPK) senses cellular energy levels and phosphorylates a variety of cellular substrates to inhibit or stimulate anabolic and catabolic processes, adjusting metabolism to energy needs. Recent studies have uncovered a crucial role of AMPK in the regulation of mitochondrial dynamics and mitophagy, further expanding its role in the control of cellular metabolism.

    • Sébastien Herzig
    •  & Reuben J. Shaw