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The human mitochondrial DNA depletion syndrome gene MPV17 encodes a non-selective channel that modulates membrane potential

Antonenkov, Vasily D.; Isomursu, Antti; Mennerich, Daniela; Vapola, Miia H.; Weiher, Hans; Kietzmann, Thomas; Hiltunen, J. Kalervo (2015-05-29)

 
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URL:
https://doi.org/10.1074/jbc.M114.608083

Antonenkov, Vasily D.
Isomursu, Antti
Mennerich, Daniela
Vapola, Miia H.
Weiher, Hans
Kietzmann, Thomas
Hiltunen, J. Kalervo
American Society for Biochemistry and Molecular Biology
29.05.2015

Antonenkov, Vasily D.; Isomursu, Antti; Mennerich, Daniela; Vapola, Miia H.; Weiher, Hans; Kietzmann, Thomas; Hiltunen, J. Kalervo

https://rightsstatements.org/vocab/InC/1.0/
© 2015 by The American Society for Biochemistry and Molecular Biology, Inc. Published in this repository with the kind permission of the publisher.
https://rightsstatements.org/vocab/InC/1.0/
doi:https://doi.org/10.1074/jbc.M114.608083
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https://urn.fi/URN:NBN:fi-fe201703152193
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Abstract

The human MPV17-related mitochondrial DNA depletion syndrome is an inherited autosomal recessive disease caused by mutations in the inner mitochondrial membrane protein MPV17. Although more than 30 MPV17 gene mutations were shown to be associated with mitochondrial DNA depletion syndrome, the function of MPV17 is still unknown. Mice deficient in Mpv17 show signs of premature aging. In the present study, we used electrophysiological measurements with recombinant MPV17 to reveal that this protein forms a non-selective channel with a pore diameter of 1.8 nm and located the channel’s selectivity filter. The channel was weakly cation-selective and showed several subconductance states. Voltage-dependent gating of the channel was regulated by redox conditions and pH and was affected also in mutants mimicking a phosphorylated state. Likewise, the mitochondrial membrane potential (Δψm) and the cellular production of reactive oxygen species were higher in embryonic fibroblasts from Mpv17−/− mice. However, despite the elevated Δψm, the Mpv17-deficient mitochondria showed signs of accelerated fission. Together, these observations uncover the role of MPV17 as a Δψm-modulating channel that apparently contributes to mitochondrial homeostasis under different conditions.

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