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J Cell Physiol. 2016 Oct;231(10):2107-14. doi: 10.1002/jcp.25319. Epub 2016 Feb 16.

Zebrafish as a Model for the Study of Chaperonopathies.

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Sbarro Institute for Cancer Research and Molecular Medicine, Philadelphia, Pennsylvania.
Department of Biology, College of Science and Technology, Temple University, Philadelphia, Pennsylvania.
Euro-Mediterranean Institute of Science and Technology (IEMEST), Palermo, Italy.
Department of Experimental Biomedicine and Clinical Neuroscience, University of Palermo, Palermo, Italy.
Department of Microbiology and Immunology, School of Medicine, University of Maryland at Baltimore and IMET, Baltimore, Maryland.


There is considerable information on the clinical manifestations and mode of inheritance for many genetic chaperonopathies but little is known on the molecular mechanisms underlying the cell and tissue abnormalities that characterize them. This scarcity of knowledge is mostly due to the lack of appropriate animal models that mimic closely the human molecular, cellular, and histological characteristics. In this article we introduce zebrafish as a suitable model to study molecular and cellular mechanisms pertaining to human chaperonopathies. Genetic chaperonopathies manifest themselves from very early in life so it is necessary to examine the impact of mutant chaperone genes during development, starting with fertilization and proceeding throughout the entire ontogenetic process. Zebrafish is amenable to such developmental analysis as well as studies during adulthood. In addition, the zebrafish genome contains a wide range of genes encoding proteins similar to those that form the chaperoning system of humans. This, together with the availability of techniques for genetic manipulations and for examination of all stages of development, makes zebrafish the organism of choice for the analysis of the molecular features and pathogenic mechanisms pertaining to human chaperonopathies. J. Cell. Physiol. 231: 2107-2114, 2016.

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