Wagenaars 2014 Abstract IOC 2014-04 Schroecken: Difference between revisions
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{{Abstract | {{Abstract | ||
|title=Wagenaars (2014) Oxygen consumption in OXPHOS-deficient cells. Mitochondr Physiol Network 19.02. | |title=Wagenaars J, Willems PHGM, Koopman WJ (2014) Oxygen consumption in OXPHOS-deficient cells. Mitochondr Physiol Network 19.02. | ||
|authors=Wagenaars J, Willems PHGM, Koopman WJ | |authors=Wagenaars J, Willems PHGM, Koopman WJ | ||
|year=2014 | |year=2014 | ||
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- Fibroblasts, myoblasts, myofibers | - Fibroblasts, myoblasts, myofibers | ||
- Intact vs. permeabilized vs. isolated mitochondria | - Intact vs. permeabilized vs. isolated mitochondria | ||
|mipnetlab=NL Nijmegen Koopman WJ | |||
}} | }} | ||
{{Labeling | {{Labeling | ||
|area=Respiration | |||
|organism=Human, Mouse | |organism=Human, Mouse | ||
|tissues=Skeletal muscle | |tissues=Skeletal muscle | ||
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|preparations=Intact cells, Permeabilized cells, Isolated Mitochondria | |preparations=Intact cells, Permeabilized cells, Isolated Mitochondria | ||
|injuries=Mitochondrial Disease; Degenerative Disease and Defect | |injuries=Mitochondrial Disease; Degenerative Disease and Defect | ||
|instruments=Oxygraph-2k | |||
}} | }} |
Revision as of 23:54, 21 March 2014
Wagenaars J, Willems PHGM, Koopman WJ (2014) Oxygen consumption in OXPHOS-deficient cells. Mitochondr Physiol Network 19.02. |
Link:
Wagenaars J, Willems PHGM, Koopman WJ (2014)
Event: IOC 2014-04 Schroecken
Mitochondria are crucially involved in cellular Ca2+ and redox homeostasis and apoptosis induction. Maintenance of mitochondrial function and integrity requires an inside-negative potential difference across the mitochondrial inner membrane. This potential is sustained by four complexes (CI-CIV) of the electron-transport chain (ETC). Together with the ATP-generating FoF1-ATPase (complex V or CV) the ETC complexes constitute the oxidative phosphorylation (OXPHOS) system. CI or NADH:ubiquinone oxidoreductase is the first and largest protein complex of the ETC and couples the oxidation of NADH to the reduction of ubiquinone. In my current research I focus on the analysis of primary skin fibroblasts from patients with mitochondrial OXPHOS disorders (including CI deficiency) and myofibers/myoblasts/myotubes from wildtype (WT) and KO animals with CI deficiency (NDUFS4-/- mice). We have observed that intact fibroblasts from patients with a mild CI deficiency display normal routine O2 consumption, whereas patient fibroblasts with a very severe CI deficiency display reduced routine O2 consumption (threshold effect). Furthermore we observed no differences in maximal (FCCP-induced) respiration in fibroblasts from patients with a mild isolated CI deficiency. To stimulate mitochondrial ATP-linked respiration, cells were treated with the hormone bradykinin during O2 consumption measurements. In this type of experiment the magnitude of the bradykinin-stimulated O2 consumption was less in fibroblasts from patients with isolated CI deficiency than in cells from a healthy individual. In this workshop I aim to increase my general knowledge of the Oroboros system and its (im)possibilities. I am particularly interested in strategies that can be applied in intact cells to investigate OXPHOS deficiencies. Moreover, the meaning and relevance of βreserve capacityβ in the context of intact cells I find particularly interesting. Cell types: - Primary patient and animal cells - Fibroblasts, myoblasts, myofibers - Intact vs. permeabilized vs. isolated mitochondria
β’ O2k-Network Lab: NL Nijmegen Koopman WJ
Labels: MiParea: Respiration
Stress:Mitochondrial Disease; Degenerative Disease and Defect"Mitochondrial Disease; Degenerative Disease and Defect" 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 Tissue;cell: Skeletal muscle Preparation: Intact cells, Permeabilized cells, Isolated Mitochondria"Isolated Mitochondria" is not in the list (Intact organism, Intact organ, Permeabilized cells, Permeabilized tissue, Homogenate, Isolated mitochondria, SMP, Chloroplasts, Enzyme, Oxidase;biochemical oxidation, ...) of allowed values for the "Preparation" property.
HRR: Oxygraph-2k