Mariusz R. Więckowski, Nencki Institute, PAS, What can we foretell from mitochondrial parameters?

event date: 19 April 2018

The Centre of New Technologies invites to a seminar by

 Prof. Mariusz R. Więckowski
Nencki Institute, PAS

What can we foretell from mitochondrial parameters?
19th of April 2018 at 12 p.m.

Venue: Centre of New Technologies, Banacha 2C, Lecture Hall 0142 (Ground floor)
Host: Marta Wiśniewska

Very often defects in mitochondrial function are associated with several pathologies, which in turn can lead to various
diseases. Improper function of the mitochondrial respiratory chain can lead to decreased ATP production, what is
often considered as a main cause of the observed symptoms. However, growing evidence has suggested a direct
relationship between development and progression of mitochondrial disorders and the presence of oxidative stress.
Increased ROS production may lead to oxidation of DNA, lipids and proteins and thus can affect fundamental cellular
processes. Whether mitochondria are the primary source of ROS production remains under debate, but mitochondria
are clearly the predominant target of the damaging effects of ROS. The aim of our studies is complex characterization
of mitochondrial respiratory chain function and the related parameters responsible for or involved in mitochondrial
defect-mediated cellular dysfunction. Our studies address mitochondrial bioenergetics and oxidative stress to
elucidate how these parameters participate in the pathogenesis of different mitochondrial disorders. We want to
determine which mitochondrial parameters are significant contributors to the development of mitochondrial disorder.
We have found that mitochondrial bioenergetic parameters, ROS production and the status of antioxidant defence
system show unique multifactorial profiles characteristic for fibroblasts from controls and patients with different
mutations in mtDNA (MTND1, MTND3, MTND5 and MTATP6) and nDNA (SURF1; SCO2, DGUOK, PDHA1 as well as in the huntingtin gen). These unique profiles of the parameters describe detected similarities, differences and
dependencies between measured parameters. We believe that such comprehensive analysis will suggest potential
therapeutic strategies in which mitochondrial physiology or ROS production is modulated to alleviate the
consequences of mitochondrial diseases.