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Possible treatment for diseases caused by mitofusin 2 deficiency

Date:
February 16, 2015
Source:
The Rockefeller University Press
Summary:
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.
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In mice, mitochondria (green) in healthy (left) and Mfn1-deficient heart muscle cells (center) are organized in a linear arrangement, but the organelles are enlarged and disorganized in Mfn2-deficient cells (right).
Credit: Mourier et al., 2015

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. Mice 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.


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Materials provided by The Rockefeller University Press. Note: Content may be edited for style and length.


Journal Reference:

  1. A. Mourier, E. Motori, T. Brandt, M. Lagouge, I. Atanassov, A. Galinier, G. Rappl, S. Brodesser, K. Hultenby, C. Dieterich, N.-G. Larsson. Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels. The Journal of Cell Biology, 2015; 208 (4): 429 DOI: 10.1083/jcb.201411100

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The Rockefeller University Press. "Possible treatment for diseases caused by mitofusin 2 deficiency." ScienceDaily. ScienceDaily, 16 February 2015. <www.sciencedaily.com/releases/2015/02/150216110710.htm>.
The Rockefeller University Press. (2015, February 16). Possible treatment for diseases caused by mitofusin 2 deficiency. ScienceDaily. Retrieved May 26, 2017 from www.sciencedaily.com/releases/2015/02/150216110710.htm
The Rockefeller University Press. "Possible treatment for diseases caused by mitofusin 2 deficiency." ScienceDaily. www.sciencedaily.com/releases/2015/02/150216110710.htm (accessed May 26, 2017).

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