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Powering up the nerves

Posted on 02/01/2019 | in 杭州夜生活 | by

Much of the activity of the nervous system is transmitted through axons, the long processes that extend out from a nerve cell's body and allow it to transmit information over long distances. In adult mammals, sensory axons may extend for meters, from the cell bodies near the spine out to the periphery of limbs, where they mediate the sensing of touch, heat, etc. Not surprisingly, given their great distance from the cell body, the ends of these axons need to act nearly independently of the control of anything happening in the cell body. The cell body can ship it raw materials, but the synapses at the far ends of the axons are otherwise on their own. HangZhou Night Net

That should provides enough background to understand some recent results pertaining to Charcot-Marie-Tooth Disease. Despite the unusual name, CMT Disease is fairly common, affecting roughly one in 2500 people, in which it causes the loss of sense and muscle control in distal limbs. A number of causative mutations have been identified; in general, these affect the production of the myelin that insulates the axons, allowing electrical charges to travel down the nerves unhindered. But the most common mutations reside in the gene MFN2, which appears to have nothing to do with myelin. Instead, MFN2 is involved with controlling the behavior of the mitochondria, the organelles that provide most of the power for cellular functions.

What goes wrong when MFN2 is mutated? The researchers put fluorescent tags on mitochondria in normal and MFN2 mutant cells, and found that the mutation caused the mitochondria to form large clusters of mitochondria. In nerve cells, these clusters of mitochondria did not migrate down the axon away from the cell body. The authors suggest that, in CMT Disease, the synapses at the far end of the axons never get enough mitochondria to supply a nearby energy source that's sufficient for them to perform their normal activity. As a result, the synapses fail, and disease results. In short, CMT Disease is the result of a broken shipping system within the axon.

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