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Available from: Stephen Carl Ekker, Oct 13, 2015
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    • "Identification and characterization of the human VWF cDNA [30–33] enabled the eventual identification of many of these pathogenic mutations as well as partial or full length sequence information in numerous mammalian species [34]. The zebrafish genome project [35] assisted in the identification of much of the vwf cDNA [15, 16], but this did not include the complete 5′ and 3′ UTRS. We have now completed cloning and characterization of the full length zebrafish vwf cDNA. "
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    ABSTRACT: von Willebrand disease (VWD) is the most common inherited human bleeding disorder and is caused by quantitative or qualitative defects in von Willebrand factor (VWF). VWF is a secreted glycoprotein that circulates as large multimers. While reduced VWF is associated with bleeding, elevations in overall level or multimer size are implicated in thrombosis. The zebrafish is a powerful genetic model in which the hemostatic system is well conserved with mammals. The ability of this organism to generate thousands of offspring and its optical transparency make it unique and complementary to mammalian models of hemostasis. Previously, partial clones of zebrafish vwf have been identified, and some functional conservation has been demonstrated. In this paper we clone the complete zebrafish vwf cDNA and show that there is conservation of domain structure. Recombinant zebrafish Vwf forms large multimers and pseudo-Weibel-Palade bodies (WPBs) in cell culture. Larval expression is in the pharyngeal arches, yolk sac, and intestinal epithelium. These results provide a foundation for continued study of zebrafish Vwf that may further our understanding of the mechanisms of VWD. Corrigendum to “Characterization of Zebrafish von Willebrand Factor Reveals Conservation of Domain Structure, Multimerization, and Intracellular Storage”
    Advances in Hematology 09/2012; 2012(3):214209. DOI:10.1155/2012/214209
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    • "Second, the optically clear body facilitates phenotypic evaluation of a number of organ systems, including, but not limited to the heart, nervous system, eyes, ears, and lateral line hair cells. The zebrafish genome is also very well characterized and manipulated (Ekker et al., 2007), for example, using morpholino oligonucleotides to knockdown molecular targets (Nasevicius and Ekker, 2000) and through the generation of transgenic zebrafish (Stuart et al., 1988; Stuart et al., 1990). The use of the zebrafish in a chemical screen was first demonstrated by Peterson et al., (2000), who identified small molecules that modulate development of the cardiovascular system, central nervous system, neural crest, and ear. "
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    ABSTRACT: The zebrafish lateral line is an efficient model system for the evaluation of chemicals that protect and damage hair cells. Located on the surface of the body, lateral line hair cells are accessible for manipulation and visualization. The zebrafish lateral line system allows rapid screens of large chemical libraries, as well as subsequent thorough evaluation of interesting compounds. In this review, we focus on the results of our previous screens and the evolving methodology of our screens for chemicals that protect hair cells, and chemicals that damage hair cells using the zebrafish lateral line.
    Hearing research 01/2012; 288(1-2):58-66. DOI:10.1016/j.heares.2012.01.009 · 2.97 Impact Factor
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    ABSTRACT: The ability to regulate gene expression in a cell-specific and temporally restricted manner provides a powerful means to test gene function, bypass the action of lethal genes, label subsets of cells for developmental studies, monitor subcellular structures, and target tissues for selective ablation or physiological analyses. The galactose-inducible system of yeast, mediated by the transcriptional activator Gal4 and its consensus UAS binding site, has proven to be a highly successful and versatile system for controlling transcriptional activation in Drosophila. It has also been used effectively, albeit in a more limited manner, in the mouse. While zebrafish has lagged behind other model systems in the widespread application of Gal4 transgenic approaches to modulate gene activity during development, recent technological advances are permitting rapid progress. Here we review Gal4-regulated genetic tools and discuss how they have been used in zebrafish as well as their potential drawbacks. We describe some exciting new directions, in large part afforded by the Tol2 transposition system, that are generating valuable new Gal4/UAS reagents for zebrafish research.
    Zebrafish 02/2008; 5(2):97-110. DOI:10.1089/zeb.2008.0530 · 1.95 Impact Factor
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