Mottier-Pavie Violaine, Megraw Timothy L
Green Center for Reproductive Biology Sciences and Department of Pharmacology, University of Texas Southwestern Medical Center, Dallas, TX 75390-9051, USA.
Mol Biol Cell. 2009 May;20(10):2605-14. doi: 10.1091/mbc.e08-11-1115. Epub 2009 Mar 25.
Cilia and flagella play multiple essential roles in animal development and cell physiology. Defective cilium assembly or motility represents the etiological basis for a growing number of human diseases. Therefore, how cilia and flagella assemble and the processes that drive motility are essential for understanding these diseases. Here we show that Drosophila Bld10, the ortholog of Chlamydomonas reinhardtii Bld10p and human Cep135, is a ubiquitous centriolar protein that also localizes to the spermatid basal body. Mutants that lack Bld10 assemble centrioles and form functional centrosomes, but centrioles and spermatid basal bodies are short in length. bld10 mutant flies are viable but male sterile, producing immotile sperm whose axonemes are deficient in the central pair of microtubules. These results show that Drosophila Bld10 is required for centriole and axoneme assembly to confer cilium motility.
纤毛和鞭毛在动物发育和细胞生理学中发挥着多种重要作用。纤毛组装或运动功能缺陷是越来越多人类疾病的病因基础。因此，纤毛和鞭毛如何组装以及驱动运动的过程对于理解这些疾病至关重要。在这里，我们表明果蝇的Bld10是莱茵衣藻Bld10p和人类Cep135的直系同源物，是一种普遍存在的中心粒蛋白，也定位于精子细胞的基体。缺乏Bld10的突变体能够组装中心粒并形成功能性中心体，但中心粒和精子细胞基体的长度较短。bld10突变体果蝇是可存活的，但雄性不育，产生的精子无运动能力，其轴丝缺乏中央微管对。这些结果表明，果蝇的Bld10是中心粒和轴丝组装以赋予纤毛运动能力所必需的。
