Wojtala 2013 Abstract MiP2013

Wojtala A, Karkucinska-Wieckowska A, Lebiedzinska M, Duszynski J, Wieckowski MR(2013) Comparative studies of reactive oxygen species production and the level of antioxidant defense system in the fibroblasts derived from patients with defined mitochondrial disorders. Mitochondr Physiol Network 18.08.

Link:

Wojtala A, Karkucinska-Wieckowska A, Lebiedzinska M, Duszynski J, Wieckowski MR (2013)

Event: MiP2013

One of the most important mitochondrial energy-providing reactions is carried out by the oxidative phosphorylation system (OXPHOS) in the mitochondrial respiratory system. OXPHOS disorders vary from fatal encephalomyopathies of early childhood, for example Leigh syndrome, to severe diseases of adulthood, like Alzheimer’s disease or Parkinson’s disease [1]. Mitochondrial respiratory deficiencies are often associated with increased reactive oxygen species (ROS) production.

The aim of the study was to determine the differences in mitochondrial bioenergetic parameters, ROS production and antioxidant enzyme levels between known mitochondrial defects. Fibroblasts derived from patients (mutations: MTND, SCO2, SURF1, MTATP6, SERAC1, TAZ and tRNALeu) with various defined mitochondrial disorders were used as a model of a self-propelling intracellular oxidative stress. Additionally we measured the level of p66Shc phosphorylation (associated with increased ROS production). In order to decrease the global and p66Shc-related ROS generation, have we tested idebenone (demonstrating antioxidant capacities) and hispidin (kinase inhibitor).

Characterization of bioenergetic parameters and ROS production showed that fibroblasts from patients demonstrate an increased ROS production and attenuated respiratory activity and have an increased status of Ser36-p66Shc phosphorylation. After treatment with hispidin or idebenone, decreased ROS production was observed in comparison with untreated cells. This could imply the involvement of p66Shc in ROS production related to the mitochondrial respiratory system. Principal component analysis of the data indicates that the particular molecular background has a unique impact on the mitochondrial dysfunctional pattern.

• Keywords: inborn mt-disease

• O2k-Network Lab: PL Warsaw Szewczyk A

Labels: MiParea: Respiration, mt-Membrane, mtDNA;mt-genetics 

Stress:RONS; Oxidative Stress"RONS; Oxidative Stress" is not in the list (Cell death, Cryopreservation, Ischemia-reperfusion, Permeability transition, Oxidative stress;RONS, Temperature, Hypoxia, Mitochondrial disease) of allowed values for the "Stress" property., Cancer; Apoptosis; Cytochrome c"Cancer; Apoptosis; Cytochrome c" is not in the list (Cell death, Cryopreservation, Ischemia-reperfusion, Permeability transition, Oxidative stress;RONS, Temperature, Hypoxia, Mitochondrial disease) of allowed values for the "Stress" property.  Organism: Human 

Preparation: Intact cells  Enzyme: Complex I, Complex II; Succinate Dehydrogenase"Complex II; Succinate Dehydrogenase" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property., Complex III, Complex IV; Cytochrome c Oxidase"Complex IV; Cytochrome c Oxidase" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property., Complex V; ATP Synthase"Complex V; ATP Synthase" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property., Marker Enzyme"Marker Enzyme" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property.  Regulation: Inhibitor, mt-Membrane potential  Coupling state: OXPHOS 

MiP2013, Prep 

Affiliations and author contributions

1 - Dept of Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland;

2 - Dept of Pathology, Children’s Memorial Heath Institute, Warsaw, Poland.

Email: a.wojtala@nencki.gov.pl

References

1. Distelmaier F, Koopman WJ, Van den Heuvel LP, Rodenburg RJ, Mayatepek E, Willems PH, Smeitink JA (2009) Mitochondrial Complex I deficiency: From organelle dysfunction to clinical disease. Brain 132: 833–842.