PUBLICATION
Animal- and In Silico Models for the study of Sarcomeric Cardiomyopathies
- Authors
- Duncker, D.J., Bakkers, J., Brundel, B.J., Robbins, J., Tardiff, J.C., Carrier, L.
- ID
- ZDB-PUB-150121-6
- Date
- 2015
- Source
- Cardiovascular research 105(4): 439-448 (Review)
- Registered Authors
- Bakkers, Jeroen
- Keywords
- none
- MeSH Terms
-
- Animals
- Cardiomyopathies*/diagnosis
- Cardiomyopathies*/genetics
- Cardiomyopathies*/metabolism
- Cardiomyopathies*/physiopathology
- Cardiomyopathies*/therapy
- Cats
- Computer Simulation*
- Dogs
- Drosophila/genetics
- Genetic Markers
- Genetic Predisposition to Disease
- Humans
- Mice, Transgenic
- Models, Animal
- Models, Cardiovascular*
- Mutation
- Phenotype
- Sarcomeres*/metabolism
- Sarcomeres*/pathology
- Zebrafish/genetics
- PubMed
- 25600962 Full text @ Cardiovasc. Res.
Citation
Duncker, D.J., Bakkers, J., Brundel, B.J., Robbins, J., Tardiff, J.C., Carrier, L. (2015) Animal- and In Silico Models for the study of Sarcomeric Cardiomyopathies. Cardiovascular research. 105(4):439-448.
Abstract
Over the past decade our understanding of cardiomyopathies has improved dramatically, due to improvements in screening and detection of gene-defects in the human genome as well as a variety of novel animal models (mouse, zebrafish and drosophila) and in silico computational models. These novel experimental tools have created a platform that is highly complementary to the naturally occurring cardiomyopathies in cats and dogs that had been available for some time. A fully integrative approach, which incorporates all these modalities, is likely required for significant steps forward in understanding the molecular underpinnings and pathogenesis of cardiomyopathies. Finally, novel technologies, including CRISPR/Cas9, which have already been proven to work in zebrafish, are currently being employed to engineer sarcomeric cardiomyopathy in larger animals, including pigs and non-human primates. In the mouse, the increased speed with which these techniques can be employed to engineer precise "knock-in" models that previously took years to make via multiple rounds of homologous recombination-based gene targeting, promises multiple and precise models of human cardiac disease for future study. Such novel genetically engineered animal models recapitulating human sarcomeric protein defects will help bridging the gap to translate therapeutic targets from small animal and in silico models to the human patient with sarcomeric cardiomyopathy.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping