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    Muscle dysfunction in a zebrafish model of Duchenne muscular dystrophy

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    Authors
    Widrick, Jeffrey J.
    Alexander, Matthew
    Sanchez, Benjamin
    Gibbs, Devin
    Kawahara, Genri
    Beggs, Alan
    Kunkel, Louis
    UMass Chan Affiliations
    Wellstone Center for FSHD
    Document Type
    Journal Article
    Publication Date
    2016-10-07
    Keywords
    disease models
    dystrophin
    muscle contraction
    muscular dystrophy
    zebrafish
    Cell Biology
    Developmental Biology
    Molecular Biology
    Molecular Genetics
    Musculoskeletal Diseases
    Nervous System Diseases
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    Link to Full Text
    https://doi.org/10.1152/physiolgenomics.00088.2016
    Abstract
    Sapje zebrafish lack the protein dystrophin and are the smallest vertebrate model of Duchenne muscular dystrophy (DMD). Their small size makes them ideal for large-scale drug discovery screens. However, the extent that sapje mimic the muscle dysfunction of higher vertebrate models of DMD is unclear. We used an optical birefringence assay to differentiate affected dystrophic sapje larvae from their unaffected siblings and then studied trunk muscle contractility at 4-7 days post fertilization. Preparation cross-sectional area (CSA) was similar for affected and unaffected larvae, yet tetanic forces of affected preparations were only 30-60% of normal. ANCOVA indicated that the linear relationship observed between tetanic force and CSA for unaffected preparations was absent in the affected population. Consequently, the average force/CSA of affected larvae was depressed 30-70%. Disproportionate reductions in twitch vs. tetanic force, and a slowing of twitch tension development and relaxation, indicated that the myofibrillar disorganization evident in the birefringence assay could not explain the entire force loss. Single eccentric contractions, in which activated preparations were lengthened 5-10%, resulted in tetanic force deficits in both groups of larvae. However, deficits of affected preparations were 3 to 5-fold greater at all strains and ages, even after accounting for any recovery. Based on these functional assessments, we conclude that the sapje mutant zebrafish is a phenotypically severe model of DMD. The severe contractile deficits of sapje larvae represent novel physiological endpoints for therapeutic drug screening.
    Source
    Physiol Genomics. 2016 Oct 7:physiolgenomics.00088.2016. doi: 10.1152/physiolgenomics.00088.2016. [Epub ahead of print] Link to article on publisher's site
    DOI
    10.1152/physiolgenomics.00088.2016
    Permanent Link to this Item
    http://hdl.handle.net/20.500.14038/50570
    PubMed ID
    27764767
    Related Resources
    Link to Article in PubMed
    ae974a485f413a2113503eed53cd6c53
    10.1152/physiolgenomics.00088.2016
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    UMass Chan Faculty and Researcher Publications
    Wellstone Center for FSHD Publications

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