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Cytoskeletal and mitochondrial pathology in immortalized R349P desmin knock-in myoblasts

Stöckmann, Philipp (2021) Cytoskeletal and mitochondrial pathology in immortalized R349P desmin knock-in myoblasts. Bachelor's, Fachhochschule Aachen, Campus Jülich.

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Abstract

For a more detailed understanding of desminopathies R349P desmin knock-in mice had previously been generated. Immortalized myoblasts that were derived from the gastrocnemius and soleus muscles of these mice were subjected to a basal characterization in the present study. The morphology, growth rate and differentiation capacity of each myoblast line were examined by light microscopy. Moreover, the desmin intermediate filament network as well as the mitochondrial content and distribution were studied in the myoblasts and differentiated myotubes by applying confocal fluorescence microscopy. Noticeable differences could be observed between homozygous myoblasts and wild-type cells. Due to the mutated desmin, a normal desmin intermediate filament network was not present in homozygous myoblasts. The mitochondria were also affected showing reduced amounts with a lack of mitochondrial networks in the R349P desmin knock-in myoblasts. The here analyzed R349P desmin knock-in myoblasts mirror essential aspects of the pathologies described in murine and human desminopathy muscle tissue and thus can serve as an additional model system to investigate the molecular pathology of desminopathies.

Item URL in elib:https://elib.dlr.de/141732/
Document Type:Thesis (Bachelor's)
Title:Cytoskeletal and mitochondrial pathology in immortalized R349P desmin knock-in myoblasts
Authors:
AuthorsInstitution or Email of AuthorsAuthor's ORCID iD
Stöckmann, PhilippUNSPECIFIEDUNSPECIFIED
Date:31 March 2021
Refereed publication:Yes
Open Access:No
Gold Open Access:No
In SCOPUS:No
In ISI Web of Science:No
Number of Pages:46
Status:Published
Keywords:Cytoskeletal, mitochondrial pathology, immortalized R349P desmin knock-in myoblasts
Institution:Fachhochschule Aachen, Campus Jülich
Department:Chemie und Biotechnologie
HGF - Research field:Aeronautics, Space and Transport
HGF - Program:Space
HGF - Program Themes:Research under Space Conditions
DLR - Research area:Raumfahrt
DLR - Program:R FR - Research under Space Conditions
DLR - Research theme (Project):R - Muscle Mechanics and Metabolism
Location: Köln-Porz
Institutes and Institutions:Institute of Aerospace Medicine > Muscle and Bone Metabolism
Deposited By: Arndt, Carina
Deposited On:15 Apr 2021 14:26
Last Modified:15 Apr 2021 14:26

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