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Imbalances in protein homeostasis caused by mutant desmin

Clemen, Christoph S. and Winter, Lilli and Unger, Andreas and Berwanger, Carolin and Spörrer, Marina and Linke, Wolfgang A. and Schröder, Rolf (2019) Imbalances in protein homeostasis caused by mutant desmin. 24th International Annual Congress of the World Muscle Society, 1 – 5 October 2019, Kopenhagen, Denmark.

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Mutations of the human desmin gene on chromosome 2q35 cause autosomal-dominant and -recessive myopathies and cardiomyopathies. For pathophysiological and therapeutic studies, we generated and characterized R349P desmin knock-in mice, which serve as patient-mimicking disease models for desminopathies. By crossbreeding our desminopathy with p53 knock-out mice, we generated immortalized desminopathy myoblast cell lines. We used these myoblasts in conjunction with skeletal muscle tissue from the desminopathy mice to study the effects of R349P mutant desmin on the ubiquitin-proteasome system, bulk autophagy, chaperone-assisted selective autophagy, and heat shock proteins.

Item URL in elib:https://elib.dlr.de/185695/
Document Type:Conference or Workshop Item (Poster)
Title:Imbalances in protein homeostasis caused by mutant desmin
AuthorsInstitution or Email of AuthorsAuthor's ORCID iDORCID Put Code
Clemen, Christoph S.UNSPECIFIEDhttps://orcid.org/0000-0002-1291-4219UNSPECIFIED
Unger, AndreasInstitute of Physiology II, University of Münster, Münster, GermanyUNSPECIFIEDUNSPECIFIED
Spörrer, MarinaCenter for Medical Physics and Technology, Friedrich-Alexander-University Erlangen-Nuremberg, 91052 Erlangen, GermanyUNSPECIFIEDUNSPECIFIED
Linke, Wolfgang A.Institute of Physiology II, University of Münster, Münster, GermanyUNSPECIFIEDUNSPECIFIED
Schröder, RolfInstitute of Neuropathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nu¨rnberg, Schwabachanlage, Erlangen, GermanyUNSPECIFIEDUNSPECIFIED
Refereed publication:Yes
Open Access:No
Gold Open Access:No
In ISI Web of Science:No
Keywords:protein homeostasis; mutant desmin; skeletal muscle myoblasts
Event Title:24th International Annual Congress of the World Muscle Society
Event Location:Kopenhagen, Denmark
Event Type:international Conference
Event Dates:1 – 5 October 2019
Organizer:European Reference Network
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: Becker, Christine
Deposited On:11 Apr 2022 10:02
Last Modified:10 Jul 2023 12:19

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