Costameric integrin and sarcoglycan protein levels are altered in a Drosophila model for Limb-girdle muscular dystrophy type 2H

Author:

Bawa Simranjot1,Gameros Samantha1,Baumann Kenny2,Brooks David S.1,Kollhoff Joseph A.1,Zolkiewski Michal1,Re Cecconi Andrea David3,Panini Nicolò4,Russo Massimo4,Piccirillo Rosanna3,Johnson David K.5,Kashipathy Maithri M.6,Battaile Kevin P.7,Lovell Scott6,Bouyain Samuel E.A.2,Kawakami Jessica2,Geisbrecht Erika R.12

Affiliation:

1. Department of Biochemistry and Molecular Biophysics, Kansas State University, Manhattan, KS 66506

2. School of Biological Sciences, University of Missouri—Kansas City, MO 64110

3. Department of Neuroscience and

4. Department of Oncology, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, 20156 Milan, Italy

5. Molecular Graphics and Modeling Laboratory, Computational Chemical Biology Core, University of Kansas, Lawrence, KS 66047

6. Protein Structure Laboratory, University of Kansas, Lawrence, KS 66047

7. NYX, New York Structural Biology Center, Upton, NY 11973

Abstract

Expression of human disease-causing LGMD2H alleles in Drosophila causes muscle degeneration and elevated levels of integrin and sarcoglycan costamere proteins, likely exacerbating disease progression.

Publisher

American Society for Cell Biology (ASCB)

Subject

Cell Biology,Molecular Biology

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