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Zebrafish: An In Vivo Model for Motile Ciliopathy

並列摘要


Cilia are highly conserved, microtubule-based organelles. The growing numbers of human diseases due to cilia dysfunction, termed ciliopathies, highlights the functional importance of cilia. Primary ciliary dyskinesia (PCD) is a genetic disorder of the motile cilia, displaying diverse clinical manifestations. With many molecular and genetic tools, zebrafish have emerged as a useful organism model, which allows the investigation of human diseases. Here, we describe the utility of zebrafish in the study of cilia and PCD. With forward and reverse genetic approaches, several novel mutations encoding essential components in motile cilia had been identified in zebrafish. These discoveries will increase our understanding toward cilia biology and have the potential to improve the diagnosis and treatment for affected individuals.

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