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.

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MiP2013, Book of Abstracts Open Access

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

Event: MiPNet18.08_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.

• O2k-Network Lab: PL_Warsaw_Szewczyk A

Labels: MiParea: Respiration, mt-Medicine, Patients  Pathology: Inherited  Stress:Oxidative stress;RONS  Organism: Human, Mouse  Tissue;cell: Fibroblast  Preparation: Intact cells  Enzyme: Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme  Regulation: Calcium, Inhibitor, Ion;substrate transport, mt-Membrane potential  Coupling state: ROUTINE  Pathway: CIV, NS  HRR: Oxygraph-2k 

MiP2013 

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: m.wieckowski@nencki.gov.pl

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