The struggle to model muscular dystrophy

Kang, Peter B.

doi:10.1002/mus.22124

Cited by 1 publication

(1 citation statement)

References 38 publications

(40 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1) and plays a pivotal role not only in transferring the contraction force from the contractile apparatus to the ECM, but also in cell signalling, membrane stabilization and Ca 2+ influx (Markert et al, 2011). Despite the detailed knowledge available on dystrophin function, no resolutive cure has been developed, partially due to the fact that dystrophin-deficient mice fail to recapitulate the clinical severity of DMD in humans (Hoffman et al, 1987; Kang, 2011). Current management of DMD is mainly based on administration of corticosteroids, which slow disease progression but do not constitute a cure.…”

Section: Modelling Dmd and Bmdmentioning

confidence: 99%

Zebrafish models flex their muscles to shed light on muscular dystrophies

Berger

Currie

2012

Disease Models &Amp; Mechanisms

View full text Add to dashboard Cite

Muscular dystrophies are a group of genetic disorders that specifically affect skeletal muscle and are characterized by progressive muscle degeneration and weakening. To develop therapies and treatments for these diseases, a better understanding of the molecular basis of muscular dystrophies is required. Thus, identification of causative genes mutated in specific disorders and the study of relevant animal models are imperative. Zebrafish genetic models of human muscle disorders often closely resemble disease pathogenesis, and the optical clarity of zebrafish embryos and larvae enables visualization of dynamic molecular processes in vivo. As an adjunct tool, morpholino studies provide insight into the molecular function of genes and allow rapid assessment of candidate genes for human muscular dystrophies. This unique set of attributes makes the zebrafish model system particularly valuable for the study of muscle diseases. This review discusses how recent research using zebrafish has shed light on the pathological basis of muscular dystrophies, with particular focus on the muscle cell membrane and the linkage between the myofibre cytoskeleton and the extracellular matrix.

show abstract

Section: Modelling Dmd and Bmdmentioning

confidence: 99%