The Charcot–Marie Tooth Disease Mutation R94Q in MFN2 Decreases ATP Production but Increases Mitochondrial Respiration under Conditions of Mild Oxidative Stress

The Charcot–Marie Tooth Disease Mutation R94Q in MFN2 Decreases ATP Production but Increases Mitochondrial Respiration under Conditions of Mild Oxidative Stress

Charcot−Marie tooth disease is a hereditary polyneuropathy caused by mutations in Mitofusin-2 (MFN2), a GTPase in the outer mitochondrial membrane involved in the regulation of mitochondrial fusion and bioenergetics. Autosomal-dominant inheritance of a R94Q mutation in MFN2 causes the axon...

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Bibliographic Details
Main Authors: Christina Wolf, Rahel Zimmermann, Osamah Thaher, Diones Bueno, Verena Wüllner, Michael K.E. Schäfer, Philipp Albrecht, Axel Methner
Format: Article
Language:English
Published: MDPI AG 2019-10-01
Series:Cells
Subjects:
oxidative stress
mfn2
mitochondria
fusion/fission
Online Access:https://www.mdpi.com/2073-4409/8/10/1289

Internet

https://www.mdpi.com/2073-4409/8/10/1289

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