Immortalised murine R349P desmin knock-in myotubes exhibit a reduced proton leak and decreased ADP/ATP translocase levels in purified mitochondria

Immortalised murine R349P desmin knock-in myotubes exhibit a reduced proton leak and decreased ADP/ATP translocase levels in purified mitochondria

Desmin gene mutations cause myopathies and cardiomyopathies. Our previously characterised R349P desminopathy mice, which carry the ortholog of the common human desmin mutation R350P, showed marked alterations in mitochondrial morphology and function in muscle tissue. By isolating skeletal muscle myo...

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Bibliographic Details
Main Authors: Carolin Berwanger, Dominic Terres, Dominik Pesta, Britta Eggers, Katrin Marcus, Ilka Wittig, Rudolf J. Wiesner, Rolf Schröder, Christoph S. Clemen
Format: Article
Language:English
Published: Elsevier 2024-06-01
Series:European Journal of Cell Biology
Subjects:
Desminopathy
Immortalised murine myoblasts
Electrical pulse stimulation
Myofibrillar maturation
Mitochondrial respiration
ADP/ATP translocase
Online Access:http://www.sciencedirect.com/science/article/pii/S0171933524000165

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http://www.sciencedirect.com/science/article/pii/S0171933524000165

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