Journal: Human Molecular Genetics
Article Title: Genetic overexpression of Serpina3n attenuates muscular dystrophy in mice
Figure Lengend Snippet: Overexpression of Serpina3n restores the abundance of sarcolemma adhesion components in two dystrophic mouse models. ( A ) Immunohistochemistry of transverse quadriceps histological sections or ( B ) western blotting from quadriceps muscle of membrane protein extracts from mice at 2–3 months of age. The genotypes of mice used are shown with the indicated antibodies. Nuclei are stained in blue with DAPI, and Ponceau was used as a western loading control. Dystr. dystrophin; Utr, utrophin; β1D-Itg, β1D-integrin; α7-Itg, α7-integrin; δ-SG, δ-sarcoglycan; α-DG, α-dystroglycan; β-DG, β-dystroglycan; β-SG, β-sarcoglycan; α-SG; α-sarcoglycan; CaV1.1, L-type calcium channel. ( C ) Quantitative protein analysis for the indicated proteins in dystrophic muscle following overexpression of Serpina3n. A total of three separate animals for each genotype were used for densitometric analysis. * P
Article Snippet: Primary antibodies used included: dystrophin [MANDYS1(3B7) 1:500]; utrophin (DSHB Mancho3-8A4 1:500); α7-integrin (Santa Cruz SC-27706 1:500); β1D-integrin (Chemicon; MAB1900, 1:200); δ-sarcoglycan (Abcam ab137101 1:1000); α-dystroglycan (Millipore 05-593 1:500); β-dystroglycan (MANDAG2 clone 7D11 1:500); β-sarcoglycan (Vector labs VP-B206 1: 500); α-sarcoglycan (DSHB IVD3(1)A9 1:500) and CaV1.1 (Pierce MA3-920 1:1000).
Techniques: Over Expression, Immunohistochemistry, Western Blot, Mouse Assay, Staining