Instytut Podstawowych Problemów Techniki
Polskiej Akademii Nauk


A. Hermann

Ostatnie publikacje
1.  Dębska-Vielhaber G., Miller I., Peeva V., Zuschratter W., Walczak J., Schreiber S., Petri S., Machts J., Vogt S., Szczepanowska J., Gellerich F.N., Hermann A., Vielhaber S., Kunz W.S., Impairment of mitochondrial oxidative phosphorylation in skin fibroblasts of SALS and FALS patients is rescued by in vitro treatment with ROS scavengers, Experimental Neurology, ISSN: 0014-4886, DOI: 10.1016/j.expneurol.2021.113620, Vol.339, pp.113620-1-10, 2021

Amyotrophic lateral sclerosis (ALS) is a devastating, rapidly progressive, neurodegenerative disorder affecting upper and lower motor neurons. Approximately 10% of patients suffer from familial ALS (FALS) with mutations in different ubiquitously expressed genes including SOD1, C9ORF72, TARDBP, and FUS. There is compelling evidence for mitochondrial involvement in the pathogenic mechanisms of FALS and sporadic ALS (SALS), which is believed to be relevant for disease. Owing to the ubiquitous expression of relevant disease-associated genes, mitochondrial dysfunction is also detectable in peripheral patient tissue. We here report results of a detailed investigation of the functional impairment of mitochondrial oxidative phosphorylation (OXPHOS) in cultured skin fibroblasts from 23 SALS and 17 FALS patients, harboring pathogenic mutations in SOD1, C9ORF72, TARDBP and FUS. A considerable functional and structural mitochondrial impairment was detectable in fibroblasts from patients with SALS. Similarly, fibroblasts from patients with FALS, harboring pathogenic mutations in TARDBP, FUS and SOD1, showed mitochondrial defects, while fibroblasts from C9ORF72 associated FALS showed a very mild impairment detectable in mitochondrial ATP production rates only. While we could not detect alterations in the mtDNA copy number in the SALS or FALS fibroblast cultures, the impairment of OXPHOS in SALS fibroblasts and SOD1 or TARDBP FALS could be rescued by in vitro treatments with CoQ10 (5 μM for 3 weeks) or Trolox (300 μM for 5 days). This underlines the role of elevated oxidative stress as a potential cause for the observed functional effects on mitochondria, which might be relevant disease modifying factors.

Słowa kluczowe:
amyotrophic lateral sclerosis, skin fibroblasts, mitochondrial dysfunction, oxidative stress

Afiliacje autorów:
Dębska-Vielhaber G. - Otto-von-Guericke University (DE)
Miller I. - inna afiliacja
Peeva V. - inna afiliacja
Zuschratter W. - inna afiliacja
Walczak J. - IPPT PAN
Schreiber S. - inna afiliacja
Petri S. - inna afiliacja
Machts J. - inna afiliacja
Vogt S. - Otto-von-Guericke University (DE)
Szczepanowska J. - Nencki Institute of Experimental Biology, Polish Academy of Sciences (PL)
Gellerich F.N. - Otto-von-Guericke University (DE)
Hermann A. - inna afiliacja
Vielhaber S. - Otto-von-Guericke University (DE)
Kunz W.S. - inna afiliacja
2.  Zeller-Plumhoff B., Robisch A.L., Pelliccia D., Longo E., Słomińska H., Hermann A., Krenkel M., Storm M., Estrin Y., Willumeit-Römer R., Salditt T., Orlov D., Nanotomographic evaluation of precipitate structure evolution in a Mg–Zn–Zr alloy during plastic deformation, Scientific Reports, ISSN: 2045-2322, DOI: 10.1038/s41598-020-72964-x, Vol.10, pp.16101-1-9, 2020

Magnesium and its alloys attract increasingly wide attention in various fields, ranging from transport to medical solutions, due to their outstanding structural and degradation properties. These properties can be tailored through alloying and thermo-mechanical processing, which is often complex and multi-step, thus requiring in-depth analysis. In this work, we demonstrate the capability of synchrotron-based nanotomographic X-ray imaging methods, namely holotomography and transmission X-ray microscopy, for the quantitative 3D analysis of the evolution of intermetallic precipitate (particle) morphology and distribution in magnesium alloy Mg–5.78Zn-0.44Zr subjected to a complex multi-step processing. A rich history of variation of the intermetallic particle structure in the processed alloy provided a testbed for challenging the analytical capabilities of the imaging modalities studied. The main features of the evolving precipitate structure revealed earlier by traditional light and electron microscopy methods were confirmed by the 3D techniques of synchrotron-based X-ray imaging. We further demonstrated that synchrotron-based X-ray imaging enabled uncovering finer details of the variation of particle morphology and number density at various stages of processing—above and beyond the information provided by visible light and electron microscopy.

Afiliacje autorów:
Zeller-Plumhoff B. - inna afiliacja
Robisch A.L. - inna afiliacja
Pelliccia D. - inna afiliacja
Longo E. - inna afiliacja
Słomińska H. - inna afiliacja
Hermann A. - inna afiliacja
Krenkel M. - inna afiliacja
Storm M. - inna afiliacja
Estrin Y. - inna afiliacja
Willumeit-Römer R. - inna afiliacja
Salditt T. - inna afiliacja
Orlov D. - inna afiliacja

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