title: What we can learn from a tadpole about ciliopathies and airway diseases: Using systems biology in Xenopus to study cilia and mucociliary epithelia.
abstract: Over the past years, the Xenopus embryo has emerged as an incredibly useful model organism for studying the formation and function of cilia and ciliated epithelia in vivo. This has led to a variety of findings elucidating the molecular mechanisms of ciliated cell specification, basal body biogenesis, cilia assembly, and ciliary motility. These findings also revealed the deep functional conservation of signaling, transcriptional, post-transcriptional, and protein networks employed in the formation and function of vertebrate ciliated cells. Therefore, Xenopus research can contribute crucial insights not only into developmental and cell biology, but also into the molecular mechanisms underlying cilia related diseases (ciliopathies) as well as diseases affecting the ciliated epithelium of the respiratory tract in humans (e.g., chronic lung diseases). Additionally, systems biology approaches including transcriptomics, genomics, and proteomics have been rapidly adapted for use in Xenopus, and broaden the applications for current and future translational biomedical research. This review aims to present the advantages of using Xenopus for cilia research, highlight some of the evolutionarily conserved key concepts and mechanisms of ciliated cell biology that were elucidated using the Xenopus model, and describe the potential for Xenopus research to address unresolved questions regarding the molecular mechanisms of ciliopathies and airway diseases.
authors.affiliation: Department of Molecular and Cell Biology; Genetics, Genomics and Development Division; Developmental and Regenerative Biology Group, University of California, Berkeley, California, 94720.
authors.forename: Peter
authors.identifier: 0000-0002-2332-6068
authors.initials: P
authors.lastname: Walentek
authors.affiliation: Salk Institute for Biological Studies, La Jolla, California, 92037.
authors.forename: Ian K
authors.initials: IK
authors.lastname: Quigley
mesh_terms: D000818:Animals; D002923:Cilia / Q000235:genetics / Q000473:pathology; D000072661:Ciliopathies / Q000235:genetics* / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D007814:Larva / Q000254:growth & development*; D008171:Lung Diseases / Q000235:genetics* / Q000473:pathology; D009079:Mucociliary Clearance / Q000235:genetics*; D015398:Signal Transduction / Q000235:genetics; D049490:Systems Biology; D014982:Xenopus laevis / Q000235:genetics
keywords: BMP signaling; Wnt signaling; gene regulatorty network; miRNA; transkription factor