PUBLICATION

Hsp90 Selectively Modulates Phenotype in Vertebrate Development

Authors
Yeyati, P.L., Bancewicz, R.M., Maule, J., and van Heyningen, V.
ID
ZDB-PUB-070409-9
Date
2007
Source
PLoS Genetics   3(3): e43 (Journal)
Registered Authors
Maule, John, van Heyningen, Veronica, Yeyati, Patricia
Keywords
Embryos, Phenotypes, Eyes, Lens (anatomy), Morpholino, Chaperone proteins, Zebrafish, Drug therapy
MeSH Terms
  • Animals
  • Animals, Genetically Modified
  • Benzoquinones/pharmacology
  • Dose-Response Relationship, Drug
  • Embryo, Nonmammalian
  • Eye Abnormalities/genetics
  • Female
  • HSP90 Heat-Shock Proteins/antagonists & inhibitors
  • HSP90 Heat-Shock Proteins/physiology*
  • Humans
  • Inheritance Patterns
  • Lactams, Macrocyclic/pharmacology
  • Male
  • Models, Biological
  • Pedigree
  • Phenotype*
  • Time Factors
  • Vertebrates/embryology*
  • Vertebrates/growth & development*
  • Zebrafish/embryology
PubMed
17397257 Full text @ PLoS Genet.
Abstract
Compromised heat shock protein 90 (Hsp90) function reveals cryptic phenotypes in flies and plants. These observations were interpreted to suggest that this molecular stress-response chaperone has a capacity to buffer underlying genetic variation. Conversely, the protective role of Hsp90 could account for the variable penetrance or severity of some heritable developmental malformations in vertebrates. Using zebrafish as a model, we defined Hsp90 inhibitor levels that did not induce a heat shock response or perturb phenotype in wild-type strains. Under these conditions the severity of the recessive eye phenotype in sunrise, caused by a pax6b mutation, was increased, while in dreumes, caused by a sufu mutation, it was decreased. In another strain, a previously unobserved spectrum of severe structural eye malformations, reminiscent of anophthalmia, microphthalmia, and nanophthalmia complex in humans, was uncovered by this limited inhibition of Hsp90 function. Inbreeding of offspring from selected unaffected carrier parents led to significantly elevated malformation frequencies and revealed the oligogenic nature of this phenotype. Unlike in Drosophila, Hsp90 inhibition can decrease developmental stability in zebrafish, as indicated by increased asymmetric presentation of anophthalmia, microphthalmia, and nanophthalmia and sunrise phenotypes. Analysis of the sunrise pax6b mutation suggests a molecular mechanism for the buffering of mutations by Hsp90. The zebrafish studies imply that mild perturbation of Hsp90 function at critical developmental stages may underpin the variable penetrance and expressivity of many developmental anomalies where the interaction between genotype and environment plays a major role.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping