2012年5月8日的Nature Communications上的一项研究描述了一个6基因的新家族，该新家族的功能是调节神经元中线粒体的运动与位置。许多神经性疾病，包括帕金森氏病和各种夏科-马里-图思病（Charcot-Marie-Tooth disease），都是由于控制线粒体转运的基因改变而引起的，其中线粒体转运是一个给细胞功能提供必需能量的过程。
这一研究指出了线粒体生物学的相关性，随着大脑大小、功能和结构的进化，线粒体转运过程变得更复杂，也可能需要另外的调节机制。正确的大脑功能是高度耗能的，然而，这种能量必须被精细地分布到整个神经元，这些神经元就是一些有分支的细胞，其分支可从大脑延伸到四肢，长达数厘米。这组基因形成线粒体的"轮子"机制部分，根据每个细胞的能量需求来调节它们的定位，就象细胞内线粒体运输的额外控制一样，还与线粒体运输调节相关的主要蛋白相互作用。
这些新蛋白还有另一个惊人特点，即它们既存在于线粒体内也存在于细胞核内，但现在还不清楚它们在细胞核内的功能，可能涉及到基因表达的调节。除了涉及大脑病理之外，这些蛋白也可能与代谢性疾病和癌症有关。（生物谷bioon.com）
doi:10.1038/ncomms1829
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The Eutherian Armcx genes regulate mitochondrial trafficking in neurons and interact with Miro and Trak2
Guillermo López-Doménech, Román Serrat, Serena Mirra, Salvatore D'Aniello, Ildiko Somorjai, Alba Abad, Nathalia Vitureira, Elena García-Arumí, María Teresa Alonso, Macarena Rodriguez-Prados, Ferran Burgaya, Antoni L. Andreu, Javier García-Sancho, Ramón Trullas, Jordi Garcia-Fernàndez, Eduardo Soriano
Brain function requires neuronal activity-dependent energy consumption. Neuronal energy supply is controlled by molecular mechanisms that regulate mitochondrial dynamics, including Kinesin motors and Mitofusins, Miro1-2 and Trak2 proteins. Here we show a new protein family that localizes to the mitochondria and controls mitochondrial dynamics. This family of proteins is encoded by an array of armadillo (Arm) repeat-containing genes located on the X chromosome. The Armcx cluster is unique to Eutherian mammals and evolved from a single ancestor gene (Armc10). We show that these genes are highly expressed in the developing and adult nervous system. Furthermore, we demonstrate that Armcx3 expression levels regulate mitochondrial dynamics and trafficking in neurons, and that Alex3interacts with the Kinesin/Miro/Trak2 complex in a Ca2+-dependent manner. Our data provide evidence of a new Eutherian-specific family of mitochondrial proteins that controls mitochondrial dynamics and indicate that this key process is differentially regulated in the brain of higher vertebrates.
