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Animal Sciences Commons

Open Access. Powered by Scholars. Published by Universities.®

Zoology

The University of Maine

2019

Muscle Disease

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Full-Text Articles in Animal Sciences

Mmp13 Inhibition Does Not Rescue Gmppb-Deficient Skeletal Muscle In Zebrafish, Sean Driscoll Apr 2019

Mmp13 Inhibition Does Not Rescue Gmppb-Deficient Skeletal Muscle In Zebrafish, Sean Driscoll

Honors College

The dystroglycan protein is one of many that attach skeletal muscle fibers to the basement membrane at the myotendinous junction. In the alpha-subunit of the dystroglycan molecule, there are sugar chains that help with the adhesion of the molecule to the basement membrane. A mutation in any gene that codes for an enzyme that adds these sugar chains can result in a form of congenital muscle disease called secondary dystroglycanopathy. One of the genes that codes for an enzyme that adds sugar chains is GMPPB and a mutation in this gene results in GMPPB-associated dystroglycanopathy. Using zebrafish as a model …