Researchers have discovered a novel role for Mitofusin 2, and the findings may point to a new treatment for patients with diseases caused by loss of the mitochondrial protein. The study appears in The Journal of Cell Biology .
Mitofusin 2 and its closely related counterpart, Mitofusin 1, are located in the outer membrane of mitochondria. Both proteins are required for mitochondrial fusion, an important maintenance function in which adjacent organelles join together and exchange contents. M
ice lacking the Mfn1 gene, which encodes Mitofusin 1, nevertheless seem perfectly healthy, but Mfn2-deficient mice die soon after birth.
Moreover, mutations in the Mfn2 gene are known to cause human diseases, including the peripheral neuropathy Charcot-Marie-Tooth type 2A. Lack of Mitofusin 2 therefore seems to affect mitochondrial function in other ways besides membrane fusion, but researchers have been unclear how.
To find out, Max Planck Institute scientist Nils-Göran Larsson and colleagues investigated mouse heart muscle cells lacking Mfn2. They found that energy metabolism in the cells was impaired compared with healthy and Mfn1-deficient cells.
They determined that the process was stalled because of reduced levels of coenzyme Q, a key component of the mitochondrial respiratory chain that generates cellular energy in the form of ATP.
In the absence of Mitofusin 2, many of the enzymes and molecules involved in the pathway that generates precursors of coenzyme Q were decreased, indicating that Mitofusin 2 is required for coenzyme Q production.
By supplementing Mfn2-deficient cells with coenzyme Q, Larsson and colleagues were able to partially restore respiratory chain function. They therefore think that coenzyme Q supplements might help treat patients with diseases caused by Mfn2 mutations.
Mourier, A., et al. 2015. J. Cell Biol. doi:10.1083/jcb.201411100.
About The Journal of Cell Biology
The Journal of Cell Biology (JCB) is published by The Rockefeller University Press. All editorial decisions on manuscripts submitted are made by active scientists in conjunction with our in-house scientific editors. JCB content is posted to PubMed Central, where it is available to the public for free six months after publication. Authors retain copyright of their published works, and third parties may reuse the content for non-commercial purposes under a creative commons license. For more information, please visit http://www.
Rita Sullivan King | EurekAlert!
Ion treatments for cardiac arrhythmia — Non-invasive alternative to catheter-based surgery
20.01.2017 | GSI Helmholtzzentrum für Schwerionenforschung GmbH
Seeking structure with metagenome sequences
20.01.2017 | DOE/Joint Genome Institute
An important step towards a completely new experimental access to quantum physics has been made at University of Konstanz. The team of scientists headed by...
Yersiniae cause severe intestinal infections. Studies using Yersinia pseudotuberculosis as a model organism aim to elucidate the infection mechanisms of these...
Researchers from the University of Hamburg in Germany, in collaboration with colleagues from the University of Aarhus in Denmark, have synthesized a new superconducting material by growing a few layers of an antiferromagnetic transition-metal chalcogenide on a bismuth-based topological insulator, both being non-superconducting materials.
While superconductivity and magnetism are generally believed to be mutually exclusive, surprisingly, in this new material, superconducting correlations...
Laser-driving of semimetals allows creating novel quasiparticle states within condensed matter systems and switching between different states on ultrafast time scales
Studying properties of fundamental particles in condensed matter systems is a promising approach to quantum field theory. Quasiparticles offer the opportunity...
Among the general public, solar thermal energy is currently associated with dark blue, rectangular collectors on building roofs. Technologies are needed for aesthetically high quality architecture which offer the architect more room for manoeuvre when it comes to low- and plus-energy buildings. With the “ArKol” project, researchers at Fraunhofer ISE together with partners are currently developing two façade collectors for solar thermal energy generation, which permit a high degree of design flexibility: a strip collector for opaque façade sections and a solar thermal blind for transparent sections. The current state of the two developments will be presented at the BAU 2017 trade fair.
As part of the “ArKol – development of architecturally highly integrated façade collectors with heat pipes” project, Fraunhofer ISE together with its partners...
19.01.2017 | Event News
10.01.2017 | Event News
09.01.2017 | Event News
20.01.2017 | Awards Funding
20.01.2017 | Materials Sciences
20.01.2017 | Life Sciences