PUBLICATION
PrPs: Proteins with a purpose: Lessons from the zebrafish
- Authors
- Málaga-Trillo, E., and Sempou, E.
- ID
- ZDB-PUB-091005-2
- Date
- 2009
- Source
- Prion 3(3): 129-133 (Review)
- Registered Authors
- Málaga-Trillo, Edward
- Keywords
- none
- MeSH Terms
-
- Animals
- Cadherins/metabolism
- Cell Adhesion
- Cell Communication
- Disease Models, Animal
- Mice
- Models, Biological
- Neurodegenerative Diseases/genetics
- Neurodegenerative Diseases/physiopathology
- Phenotype
- Phosphorylation
- Prion Diseases/genetics
- Prion Diseases/physiopathology*
- Prions/genetics*
- Prions/physiology*
- Protein Isoforms
- Zebrafish
- PubMed
- 19786844 Full text @ Prion
Citation
Málaga-Trillo, E., and Sempou, E. (2009) PrPs: Proteins with a purpose: Lessons from the zebrafish. Prion. 3(3):129-133.
Abstract
The best-known attribute of the prion protein (PrP) is its tendency to misfold into a rogue isoform. Much less understood is how this misfolded isoform causes deadly brain illnesses. Neurodegeneration in prion disease is often seen as a consequence of abnormal PrP function yet, amazingly little is known about the normal, physiological role of PrP. In particular, the absence of obvious phenotypes in PrP knockout mice has prevented scientists from answering this important question. Using knockdown approaches, we previously produced clear PrP loss-of-function phenotypes in zebrafish embryos. Analysis of these phenotypes revealed that PrP can modulate E-cadherin-based cell-cell adhesion, thereby controlling essential morphogenetic cell movements in the early gastrula. Our data also showed that PrP itself can elicit homophilic cell-cell adhesion and trigger intracellular signaling via Src-related kinases. Importantly, these molecular functions of PrP are conserved from fish to mammals. Here we discuss the use of the zebrafish in prion biology, and how it may advance our understanding of the roles of PrP in health and disease.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping