Department of Anatomy and Developmental Biology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kjiicho, Kamighyo-ku, Kyoto 602-8566, Japan.
Differentiation. 2012 Feb;83(2):S23-9. doi: 10.1016/j.diff.2011.11.009. Epub 2011 Dec 7.
Motile cilia and flagella are organelles, which function in cell motility and in the transport of fluids over the surface of cells. Motility defects often result in a rare human disease, primary ciliary dyskinesia (PCD). Cell motility depends on axonemal dynein, a molecular motor that drives the beating of cilia and flagella. The dyneins are composed of multiple subunits, which are thought to be preassembled in the cytoplasm before they are transported into cilia and flagella. Axonemal dyneins have been extensively studied in Chlamydomonas. In addition, analyses of human PCDs over the past decade, together with studies in other model animals, have identified the conserved components required for dynein assembly. Recently also, the first cytoplasmic component of dynein assembly, kintoun (ktu), was elucidated through the analysis of a medaka mutant in combination with human genetics and cell biology and biochemical studies of Chlamydomonas. The components of dynein and the proteins involved in its cytoplasmic assembly process are discussed.
纤毛和鞭毛是细胞运动和细胞表面液体运输的细胞器。运动缺陷常导致一种罕见的人类疾病，即原发性纤毛运动障碍（PCD）。细胞运动依赖于轴丝动力蛋白，这是一种分子马达，可驱动纤毛和鞭毛的摆动。动力蛋白由多个亚基组成，这些亚基被认为在运输到纤毛和鞭毛之前就在细胞质中预先组装。轴丝动力蛋白在衣藻中得到了广泛研究。此外，过去十年对人类 PCD 的分析，以及对其他模式动物的研究，确定了动力蛋白组装所需的保守成分。最近，通过分析一种与人类遗传学和细胞生物学以及衣藻的生化研究相结合的斑马鱼突变体，阐明了动力蛋白组装的第一个细胞质成分，即 kintoun（ktu）。讨论了动力蛋白的组成及其细胞质组装过程中涉及的蛋白质。
