Marshall Wallace F, Nonaka Shigenori
Department of Biochemistry and Biophysics, University of California San Francisco, 600 16th St., San Francisco, California 94143, USA.
Curr Biol. 2006 Aug 8;16(15):R604-14. doi: 10.1016/j.cub.2006.07.012.
Cilia are microtubule-based organelles that project like antennae from the surface of most cells in the body. Motile cilia move fluid past cells, for example mucus in the airway. Non-motile primary cilia, however, transduce a multitude of sensory stimuli, including chemical concentrations of growth factors, hormones, odorants, and developmental morphogens, as well as osmolarity, light intensity, and fluid flow. Cilia have evolved a complex ultrastructure to accommodate these diverse functions, and an extensive molecular machinery has developed to support the assembly of these organelles. Defects in the cilia themselves, or the machinery required to assemble them, lead to a broad spectrum of human disease symptoms, including polycystic kidney disease, nephronophthisis, hydrocephalus, polydactyly, situs inversus, retinal degeneration, and obesity. While these diseases highlight the pivotal roles of cilia in physiology and development, the mechanistic link between cilia, physiology, and disease remains unclear.
纤毛是以微管为基础的细胞器，像触角一样从身体大多数细胞的表面伸出。运动性纤毛使液体流过细胞，例如气道中的黏液。然而，非运动性初级纤毛可转导多种感觉刺激，包括生长因子、激素、气味剂和发育形态发生素的化学浓度，以及渗透压、光强度和流体流动。纤毛进化出了复杂的超微结构以适应这些多样的功能，并且已经发展出广泛的分子机制来支持这些细胞器的组装。纤毛本身或组装它们所需的机制存在缺陷会导致一系列广泛的人类疾病症状，包括多囊肾病、肾单位肾痨、脑积水、多指畸形、内脏反位、视网膜变性和肥胖。虽然这些疾病凸显了纤毛在生理学和发育中的关键作用，但纤毛、生理学和疾病之间的机制联系仍不清楚。
