Kamienieva 2023 Biochim Biophys Acta Mol Basis Dis
| Kamienieva I, Charzyńska A, Duszyński J, Malińska D, Szczepanowska J (2023) In search for mitochondrial biomarkers of Parkinson's disease: Findings in parkin-mutant human fibroblasts. https://doi.org/10.1016/j.bbadis.2023.166787 |
» Biochim Biophys Acta Mol Basis Dis 1869:166787. PMID: 37302428 Open Access
Kamienieva Iryna, Charzynska Agata, Duszynski Jerzy, Malinska Dominika, Szczepanowska Joanna (2023) Biochim Biophys Acta Mol Basis Dis
Abstract: Most cases of Parkinson's disease (PD) are idiopathic, with unknown aetiology and genetic background. However, approximately 10 % of cases are caused by defined genetic mutations, among which mutations in the parkin gene are the most common. There is increasing evidence of the involvement of mitochondrial dysfunction in the development of both idiopathic and genetic PD. However, the data on mitochondrial changes reported by different studies are inconsistent, which can reflect the variability in genetic background of the disease. Mitochondria, as a plastic and dynamic organelles, are the first place in the cell to respond to external and internal stress. In this work, we characterized mitochondrial function and dynamics (network morphology and turnover regulation) in primary fibroblasts from PD patients with parkin mutations. We performed clustering analysis of the obtained data to compare the profiles of mitochondrial parameters in PD patients and healthy donors. This allowed to extract the features characteristic for PD patients fibroblasts, which were a smaller and less complex mitochondrial network and decreased levels of mitochondrial biogenesis regulators and mitophagy mediators. The approach we used allowed a comprehensive characteristics of elements common for mitochondrial dynamics remodelling accompanying pathogenic mutation. This may be helpful in the deciphering key pathomechanisms of the PD disease. • Keywords: Mitochondria, Parkin, Parkinson's disease • Bioblast editor: Plangger M • O2k-Network Lab: PL Warsaw Szewczyk A
Labels: MiParea: Respiration, Patients
Pathology: Parkinson's
Organism: Human Tissue;cell: Fibroblast Preparation: Intact cells
Coupling state: LEAK, ROUTINE, ET
HRR: Oxygraph-2k
2023-06
