PUBLICATION
Fishing for causes and cures of motor neuron disorders
- Authors
- Patten, S.A., Armstrong, G.A., Lissouba, A., Kabashi, E., Parker, J.A., Drapeau, P.
- ID
- ZDB-PUB-140629-6
- Date
- 2014
- Source
- Disease models & mechanisms 7: 799-809 (Review)
- Registered Authors
- Drapeau, Pierre
- Keywords
- ALS, Drug discovery, HSP, Motor neuron disorders, SMA, Zebrafish
- MeSH Terms
-
- Animals
- Disease Models, Animal
- Drug Evaluation, Preclinical
- Humans
- Motor Neuron Disease/drug therapy*
- Motor Neuron Disease/etiology*
- Motor Neuron Disease/pathology
- Signal Transduction
- Zebrafish/metabolism*
- PubMed
- 24973750 Full text @ Dis. Model. Mech.
Citation
Patten, S.A., Armstrong, G.A., Lissouba, A., Kabashi, E., Parker, J.A., Drapeau, P. (2014) Fishing for causes and cures of motor neuron disorders. Disease models & mechanisms. 7:799-809.
Abstract
Motor neuron disorders (MNDs) are a clinically heterogeneous group of neurological diseases characterized by progressive degeneration of motor neurons, and share some common pathological pathways. Despite remarkable advances in our understanding of these diseases, no curative treatment for MNDs exists. To better understand the pathogenesis of MNDs and to help develop new treatments, the establishment of animal models that can be studied efficiently and thoroughly is paramount. The zebrafish (Danio rerio) is increasingly becoming a valuable model for studying human diseases and in screening for potential therapeutics. In this Review, we highlight recent progress in using zebrafish to study the pathology of the most common MNDs: spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS) and hereditary spastic paraplegia (HSP). These studies indicate the power of zebrafish as a model to study the consequences of disease-related genes, because zebrafish homologues of human genes have conserved functions with respect to the aetiology of MNDs. Zebrafish also complement other animal models for the study of pathological mechanisms of MNDs and are particularly advantageous for the screening of compounds with therapeutic potential. We present an overview of their potential usefulness in MND drug discovery, which is just beginning and holds much promise for future therapeutic development.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping