关键词: 纤毛, 微管蛋白蛋白质翻译后修饰, 微管蛋白密码, 纤毛病

Abstract:

Cilia are hair-like structures protruding from the surface of eukaryotic cells, composed of a microtubule-based cytoskeleton and bounded by ciliary membrane, which play a crucial role in organ development and homeostasis maintenance in organisms. The ciliary tubulin undergoes several highly conserved posttranslational modifications (PTMs) including acetylation, glutamylation, glycylation and detyrosination. The studies indicate that ciliary tubulin PTMs are adequate to precisely regulate the fine tuning of assembly/disassembly, maintenance, motility, and signaling of cilia. Dysregulated ciliary tubulin PTMs leads to ciliary dysfunction, strongly implicated in human disorders including ciliopathies. Here, we review the current understanding how ciliary tubulin PTMs regulate cilia formation and function, with a focus on their association with human disorders, which may provide novel avenues for therapeutic intervention in ciliopathies

Key words: Cilia, Tubulin Posttranslational modifications, Tubulin code, Ciliopathies