Gainutdinov 2022 MitoFit
|Gainutdinov T, Debska-Vielhaber G, Gizatullina Z, Vielhaber S, Orynbayeva Z, Gellerich FN (2022) Impaired Ca2+ signalling as an indicator of disturbed mitochondrial function in fibroblasts from patients with sporadic and familial ALS. https://doi.org/10.26124/mitofit:2022-0015.v2|
» MitoFit Preprints 2022.15.v.2
Amyotrophic lateral sclerosis (ALS) is a progressive, devastating, neurodegenerative disorder affecting upper and lower motor neurons. Common mechanisms of ALS pathogenesis are believed to be the disturbance of calcium homeostasis in the cell and dysfunction of mitochondria. Both factors mutually influence each other. As a result, chronic mitochondrial energy stress impairs fine cellular signaling and transport processes, leading to degeneration of motor neurons. In the current study we comparatively evaluated the cytosolic Ca2+ in healthy and ALS fibroblasts. We found that the mitochondrial calcium capacity in fibroblasts obtained from patients with sporadic (sALS) and familial (fALS) ALS differs between two subtypes and from that in healthy individuals. The changes of [Ca2+]cyt dynamics in ALS fibroblasts could be almost completely rescued by treatment with antioxidants (Trolox and CoQ10). These data confirm an important role of oxidative stress as a causative factor of mitochondrial dysfunction in ALS.
• Keywords: ALS, CoQ10, cellular Ca2+ homeostasis, histamine, mitochondria, Trolox • Bioblast editor: Tindle-Solomon L, Cecatto C • O2k-Network Lab: DE Bonn Kunz WS, DE Magdeburg Debska-Vielhaber G, DE Magdeburg Gellerich FN
Labels: MiParea: Patients Pathology: Inherited, Neurodegenerative
Organism: Human Tissue;cell: Fibroblast Preparation: Intact cells