Wieckowski 2013 Abstract MiP2013

Wieckowski MR, Karkucinska-Wieckowska A, Wojtala A, Lebiedzinska M, Pronicki M, Duszynski J (2013) Mitochondrial bioenergetic parameters, reactive oxygen species production and the status of antioxidant defense system can be used to differentiate mitochondrial defects studied in the fibroblasts from patients with various mitochondrial disorders. Mitochondr Physiol Network 18.08.

Link:

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

Event: MiP2013

Defects in the mitochondrial respiratory system are often associated with mitochondrial dysfunction and increased reactive oxygen species (ROS) production within the cell. The aim of our studies was to determine the differences in the mitochondrial bioenergetic parameters, ROS production and antioxidant enzymes status profiles between different types of mitochondrial defects.

Fibroblasts derived from patients with defined mitochondrial disorders (mutations in the genes of subunits Complex I, SCO2, SURF1, MTATP6, SERAC1, TAZZ and tRNA^Leu) have been studied. Bioenergetic parameters, ROS production and the level of individual antioxidant enzymes have been estimated. Finally, the multiparameter statistical analysis has been performed.

Anomalies in the bioenergetic parameters, modification of the antioxidant enzymes levels as well as enhancement of intracellular ROS confirmed the occurrence of oxidative stress in the fibroblasts. Principal component analysis showed that individual defects were grouped in separate clusters. This indicates that mitochondrial defects in the patients’ fibroblasts are characterized by a unique profile of important parameters of cellular bioenergetics and ROS homeostasis as well as that the different molecular background has a unique impact on the mitochondrial and antioxidant defense system dysfunctional pattern.

This approach may open new possibility to use the proposed set of mitochondrial parameters and comparative analysis in the studies essential for distinguishing the molecular background of mitochondrial defect.

• Keywords: Ion homeostasis, inborn mt-disease

• O2k-Network Lab: PL_Warsaw_Szewczyk A

Labels: MiParea: Respiration 

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, Mouse 

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., Inner mtMembrane Transporter"Inner mtMembrane Transporter" 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: Calcium, Inhibitor, mt-Membrane potential, O2"O2" is not in the list (Aerobic glycolysis, ADP, ATP, ATP production, AMP, Calcium, Coupling efficiency;uncoupling, Cyt c, Flux control, Inhibitor, ...) of allowed values for the "Respiration and regulation" property.  Coupling state: OXPHOS 

MiP2013, Prep 

Affiliations and author contributions

1 - Dept Pathology, The Children’s Memorial Health Institute, Warsaw, Poland;

2 - Dept Biochemistry, Nencki Institute of Experimental Biology, Warsaw, Poland

Email: [email protected]

Supported by the Statutory Founding from Nencki Institute of Experimental Biology, MNiSW nr W100/HFSC/2011 and Internal Projects of CMHI 125/2012.