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SUMMARY:Management of mitochondrial proteins: sort or destroy  - Professor
  Agnieszka Chacinska
DTSTART:20190117T150000Z
DTEND:20190117T160000Z
UID:TALK118078@talks.cam.ac.uk
CONTACT:Hannah Burns
DESCRIPTION:Mitochondria are multifunctional organelle\, primarily involve
 d in fundamental biological process\, respiration. The efficient functioni
 ng of mitochondria depends on the proper transport\, sorting and assembly 
 of mitochondrial proteins that originate either from nuclear or mitochondr
 ial genomes. Both nuclear and mitochondrial genome defects resulting in mu
 tated proteins have been implicated in variety of mitochondrial diseases. 
 The nuclear encoded proteins make up for the large majority of the protein
 s involved in formation of mitochondria including the respiratory chain co
 mplexes. The ubiquitin - proteasome system (UPS) in cytosol is largely inv
 olved in degradation of cellular proteins and maintaining protein homeosta
 sis. By multiple lines of evidence we have demonstrated the contribution o
 f UPS to mitochondrial IMS protein quality control. The UPS degrades a por
 tion of mitochondrial proteins including mislocalised proteins\, in both y
 east and mammalian systems. Furthermore\, mislocalization of mitochondrial
  proteins increases the ability of the proteasome to degrade cellular prot
 eins. Thus\, the UPS constitutes an important factor that influences mitoc
 hondrial proteome in physiology and links mitochondrial status with regula
 tion of cellular protein homeostasis. Malfunctioning of mitochondria is a 
 cause of devastating mitochondrial disease. Pathologic variants of mitocho
 ndrial proteins can be mistargeted and fully degraded by the proteasome be
 fore they reach their final destination inside mitochondria. Inhibition of
  proteasomal degradation by commonly used proteasome inhibitors leads to r
 escue of proteins and their import into mitochondria. Thus\, UPS inhibitio
 n can provide a benefit to malfunctioning mitochondria. We propose that ta
 rgeting the UPS should be considered as a therapeutic strategy for mitocho
 ndrial diseases.
LOCATION:Sackler Lecture Theatre (Level 7) Wellcome Trust/MRC Building\, C
 ambridge Biomedical Campus
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