PUBLICATION

Zebrafish as a Model for the Study of Chaperonopathies

Authors
Bellipanni, G., Cappello, F., Scalia, F., de Macario, E.C., Macario, A.J., Giordano, A.
ID
ZDB-PUB-160128-5
Date
2016
Source
Journal of Cellular Physiology   231(10): 2107-14 (Review)
Registered Authors
Bellipanni, Gianfranco
Keywords
CCT, Hsp, Hsp40, Hsp60, Hsp70, chaperoning system, chaperonins, chaperonopathies, chaperonotherapy, development, heat-shock proteins, molecular chaperones, mutation, zebrafish
MeSH Terms
  • Animals
  • Genetic Predisposition to Disease/genetics*
  • Genetic Testing/methods
  • Humans
  • Models, Animal*
  • Molecular Chaperones/genetics*
  • Mutation/genetics
  • Zebrafish/genetics*
  • Zebrafish/growth & development
PubMed
26812965 Full text @ J. Cell. Physiol.
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. This article is protected by copyright. All rights reserved.
Genes / Markers
Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping