Pubblicazione scientifica

Fishing for causes and cures of motor neuron disorders

FILONE TECNOLOGICO	TEMA
produzione e cattura

RIASSUNTO

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.

DATA

Data di pubblicazione: 01/07/2014

AUTORI

PATTEN SHUNMOOGUM A
ARMSTRONG GARY A B
LISSOUBA ALEXANDRA
KABASHI EDOR
PARKER J ALEX
DRAPEAU PIERRE

ENTE DI AFFERENZA

DEPT NEUROSCIENCE FRQS GROUPE DE RECHERCHE SUR LE SYS NERVEUX CENTRAL & CRCHUM UNIV MONTREAL MONTREAL QC H3A 2B4 CANADA
INST CERVEAU & MOELLE EPINIERE CTR RECHERCHE CHU PITIE SALPETRIERE 75013 PARIS FRANCE

RIVISTA

Disease Models & Mechanisms, 7(7), 799-809

IMPACT FACTOR

3,87

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