title: Fishing for causes and cures of motor neuron disorders.
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.
authors: Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
authors: Shunmoogum A
authors: SA
authors: Patten
authors: Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
authors: Gary A B
authors: GA
authors: Armstrong
authors: Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
authors: Alexandra
authors: A
authors: Lissouba
authors: Institut du Cerveau et de la Moelle Épinière, Centre de Recherche, CHU Pitié-Salpétrière, 75013 Paris, France.
authors: Edor
authors: E
authors: Kabashi
authors: Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada.
authors: J Alex
authors: JA
authors: Parker
authors: Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montréal, Montréal, QC H3A 2B4, Canada. p.drapeau@umontreal.ca.
authors: Pierre
authors: P
authors: Drapeau
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D004353:Drug Evaluation, Preclinical; D006801:Humans; D016472:Motor Neuron Disease / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D015398:Signal Transduction; D015027:Zebrafish / Q000378:metabolism*
keywords: ALS; Drug discovery; HSP; Motor neuron disorders; SMA; Zebrafish