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 — 2022-12-09 published in Bioenerg Commun 2022.18.

» MitoFit Preprints 2022.15.v.2

Impaired Ca²⁺ signalling as an indicator of disturbed mitochondrial function in fibroblasts from patients with sporadic and familial ALS

Gainutdinov Timur, Debska-Vielhaber Grazyna, Gizatullina Zemfira, Vielhaber Stefan, Orynbayeva Zulfiya, Gellerich Frank Norbert (2022) MitoFit Prep

Abstract:

Version 2 (v2) 2022-08-16 https://doi.org/10.26124/mitofit:2022-0015.v2

Version 1 (v1) 2022-04-21 https://doi.org/10.26124/mitofit:2022-0015- »Link to all versions«

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

ORCID: Gainutdinov Timur, Debska-Vielhaber Grazyna, Gizatullina Zemfira, Vielhaber Stefan, Orynbayeva Zulfiya, Gellerich Frank Norbert

Labels: MiParea: Patients Pathology: Inherited, Neurodegenerative

Organism: Human Tissue;cell: Fibroblast Preparation: Intact cells

Regulation: Calcium