Cellular mechanisms handling failed mitochondrial protein translocation events

Project Leader: Piotr Brągoszewski, PhD Project period: 2017 - 2020
Project funding: First TEAM, FNP
Project description:
Mitochondria are essential organelles in eukaryotic cells. Most mitochondrial proteins originate from the cytosol and require active import into the organelle. Protein import is not always error-free even under physiological conditions. Translocation failure can result in translocases clogging by misfolded proteins in transit. The toxicity of misfolded proteins and mitochondrial dysfunction are pivotal factors in many pathological conditions including neurodegenerative disorders, such as Alzheimer’s and Parkinson’s diseases. Our current understanding of the import process is well detailed but little is known about quality control mechanisms that resolve failed protein translocation events. We hypothesize that specific molecular quality control machinery exists in the cell to resolve protein translocase clogging. The project’s aim is to uncover and characterise this response at the molecular level, and to understand the role of this quality control mechanism in physiology and pathology.
Laboratory of Mitochondrial Biogenesis