Zebrafish as a Model for the Study of Chaperonopathies

Francesco Cappello, Federica Scalia, Gianfranco Bellipanni, Francesco Cappello, Everly Conway De Macario, Alberto J.L. Macario, Antonio Giordano, Antonio Giordano

Risultato della ricerca: Articlepeer review

4 Citazioni (Scopus)

Abstract

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. 9999: 1-8, 2016.
Lingua originaleEnglish
pagine (da-a)2107-14-2114
Numero di pagine8
RivistaJournal of Cellular Physiology
Volume231
Stato di pubblicazionePublished - 2016

All Science Journal Classification (ASJC) codes

  • Physiology
  • Clinical Biochemistry
  • Cell Biology

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