Distal axons, remote from their cell bodies and nuclei, must survive the lifetime of an organism. Recent studies have provided compelling evidence that proteins are locally synthesized in healthy, mature central nervous system axons and presynaptic terminals in vivo. Presynaptic, mitochondrial and ribosomal proteins are locally synthesized in most adult axons of diverse cell types, linking local translation to axon function and survival. Accordingly, inhibiting the intra-axonal translation of key mRNAs or the function of their translational regulators causes dying-back axon degeneration, and human mutations in RNA metabolic pathways are increasingly being associated with neurodegenerative diseases that accompany axon degeneration. Here, we summarize recent relevant findings in a highly simplified 'RNA operon'-based model and discuss open questions and future directions.

中文翻译：

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长期维持轴突健康和功能的局部mRNA翻译。

远离细胞体和细胞核的远端轴突必须在生物体的生命周期中生存。最近的研究提供了令人信服的证据，证明蛋白质是在体内健康，成熟的中枢神经系统轴突和突触前末端局部合成的。突触前蛋白，线粒体蛋白和核糖体蛋白在各种细胞类型的大多数成年轴突中局部合成，将局部翻译与轴突功能和存活联系起来。因此，抑制关键mRNA的轴突内翻译或其翻译调节子的功能导致垂死的轴突变性，并且RNA代谢途径中的人突变与轴突变性伴随的神经退行性疾病越来越相关。在这里，我们在高度简化的“ RNA操纵子”中总结了最近的相关发现。