Barry Devin M, Millecamps Stephanie, Julien Jean-Pierre, Garcia Michael L
Division of Biological Sciences, University of Missouri-Columbia, 1201 East Rollins Street, Columbia, MO 65211, USA.
Exp Cell Res. 2007 Jun 10;313(10):2110-20. doi: 10.1016/j.yexcr.2007.03.011. Epub 2007 Mar 21.
Revealing the mechanisms by which neurofilament transport and turnover are regulated has proven difficult over the years but recent studies have given new insight into these processes. Mature neurofilament fibers may incorporate a fourth functional subunit, alpha-internexin, as new evidence suggests. Recent findings have made the role of phosphorylation in regulating neurofilament transport velocity controversial. Kinesin and dynein may transport neurofilaments in slow axonal transport as they have been found to associate with neurofilaments. Neurofilament transport and turnover rates may be reduced depending on the existing stationary neurofilament network. Finally, mutations in neurofilament light that have been linked to Charcot-Marie-Tooth disease as well as other neurofilament abnormalities in human disease are discussed.
多年来，揭示神经丝运输和周转的调节机制一直颇具难度，但最近的研究为这些过程提供了新的见解。新证据表明，成熟的神经丝纤维可能包含第四个功能亚基——α-中间丝蛋白。最近的研究结果使磷酸化在调节神经丝运输速度中的作用存在争议。驱动蛋白和动力蛋白可能在轴突慢速运输中运输神经丝，因为它们已被发现与神经丝相关联。神经丝的运输和周转率可能会根据现有的静止神经丝网络而降低。最后，讨论了与夏科-马里-图斯病相关的神经丝轻链突变以及人类疾病中的其他神经丝异常情况。
