Journal: The American Journal of Pathology
Article Title: The D2.B10- Dmd mdx /J Mouse Model of Duchenne Muscular Dystrophy Exhibits a Severe Mitochondrial Deficiency Not Observed in the C57BL/10ScSn- Dmd mdx /J Mouse
doi: 10.1016/j.ajpath.2025.09.005
Figure Lengend Snippet: Relative protein expression changes to the latent transforming growth factor (TGF)-β–binding protein 4 (LTBP4) pathway and annexin A6 (ANXA6). LTBP4 is a known genetic modifier in human patients with Duchenne muscular dystrophy and impacts TGF-β signaling. TGF-β acts on SMAD4, which directly interacts with mitochondrial cytochrome c oxidase subunit 2 (MTCO2) and can cause mitochondrial fragmentation via dynamin-related protein 1 (DRP1). A – L: All proteins were assessed for expression levels ( A – F ) relative to total protein ( G – L ). H: TGF-β levels were elevated in D2-WT mice compared with B10-WT mice, and complex IV subunit 2 (MTCO2) was elevated in the D2- mdx mice compared with D2-WT. ∗ P < 0.05, ∗∗ P < 0.01.
Article Snippet: Antibodies against latent TGF-β–binding protein 4 (LTBP4; Novus Biologicals, Centennial, CO; NBP2-43671; 1:500), TGF-β (Novus Biologicals; NBP2-46108; 1:2000), annexin A6 (ANXA6; ProteinTech, Rosemont, IL; 68086-1-Ig; 1:2000), mitochondrial cytochrome c oxidase subunit 2 (MTCO2; Thermo Fisher; A-6404; 1:500), SMAD4 (ProteinTech; 10231-1-AP; 1:100), dynamin-related protein 1 (DRP1; ProteinTech; 12957-1-AP; 1:1000), and Total OXPHOS Rodent WB Antibody Cocktail (Abcam; ab110413; 1:25,000) were used.
Techniques: Expressing, Binding Assay