Control of mitochondrial integrity in ageing and disease

Radek Szklarczyk*, Marco Nooteboom, Heinz D. Osiewacz

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Various molecular and cellular pathways are active in eukaryotes to control the quality and integrity of mitochondria. These pathways are involved in keeping a 'healthy' population of this essential organelle during the lifetime of the organism. Quality control (QC) systems counteract processes that lead to organellar dysfunction manifesting as degenerative diseases and ageing. We discuss disease-and ageing-related pathways involved in mitochondrial QC: mtDNA repair and reorganization, regeneration of oxidized amino acids, refolding and degradation of severely damaged proteins, degradation of whole mitochondria by mitophagy and finally programmed cell death. The control of the integrity of mtDNA and regulation of its expression is essential to remodel single proteins as well as mitochondrial complexes that determine mitochondrial functions. The redundancy of components, such as proteases, and the hierarchies of the QC raise questions about crosstalk between systems and their precise regulation. The understanding of the underlying mechanisms on the genomic, proteomic, organellar and cellular levels holds the key for the development of interventions for mitochondrial dysfunctions, degenerative processes, ageing and age-related diseases resulting from impairments of mitochondria.
Original languageEnglish
Article number20130439
JournalPhilosophical Transactions of the Royal Society B-biological Sciences
Issue number1646
Publication statusPublished - 5 Jul 2014


  • mitochondria
  • quality control
  • disease
  • ageing


Dive into the research topics of 'Control of mitochondrial integrity in ageing and disease'. Together they form a unique fingerprint.

Cite this