Journal: bioRxiv

Article Title: AAV-Delivered RNAi Targeting Mutant LDB3 Prevents and Reverses Myofibrillar Myopathy through Mechanosignaling Restoration

doi: 10.64898/2026.03.28.715031

Figure Lengend Snippet: Representative IF images of LDB3 and filamin C in PFA perfused longitudinal TA muscle sections from advanced-stage, (5-month-old) Ldb3 Ala165Val/+ mice treated with sh-SC, sh16, or sh10 ( A and B) for one month (upper panel) or three months (bottom panel) (n = 4-6 per group). (C and D) Quantification of muscle fibers containing sarcoplasmic filamin C aggregates in sh-SC control and shRNA-treated groups. Bar scatter plots represent the percentage of fibers exhibiting filamin C aggregates, quantified from at least five randomly selected areas (> 50 fibers per muscle) from immunostained TA sections (n = 3 per group). (E and F) Representative IF images of BAG3 and HSPA8 in longitudinal TA cryosections from advanced-stage, (5-month-old) Ldb3 Ala165Val/+ mice treated with sh-SC, sh16, or sh10 for 1 month (upper panels) or 3 months (lower panels) (n = 4-6 per group). Data are expressed as mean ± SEM, with statistical significance denoted by ****P < 0.0001, determined using an unpaired, two-tailed Student’s t-test. Scale bars represent 50 µm (A and B) and 20 µm (E and F).

Article Snippet: Sections were incubated overnight at 4 °C with primary antibodies against LDB3, filamin C, BAG3, HSPA8, and EGFP (Cell Signaling Technology, ab184601); antibody details and dilutions were as described previously.

Techniques: Control, shRNA, Two Tailed Test

Journal: bioRxiv

Article Title: AAV-Delivered RNAi Targeting Mutant LDB3 Prevents and Reverses Myofibrillar Myopathy through Mechanosignaling Restoration

doi: 10.64898/2026.03.28.715031

Figure Lengend Snippet: (A and C) Representative IF images of LDB3 and filamin C in PFA perfused longitudinal TA muscle sections from early-stage (3-month-old) Ldb3 Ala165Val/+ mice treated with sh-SC, sh16 (A), or sh10 (C) for one month (upper panel) or three months (lower panel) (n = 4-6 per group). (B and D) Quantification of muscle fibers containing sarcoplasmic filamin C aggregates in sh-SC and shRNA-treated groups corresponding to panels (A) and (C). Bar-scatter plots represent the percentage of fibers exhibiting filamin C aggregates, quantified from at least five randomly selected fields (>50 fibers per muscle) per section (n = 3 per group). (E and F) Representative IF images of BAG3 and HSPA8 in longitudinal TA cryosections from early-stage, (3-month-old) Ldb3 Ala165Val/+ mice treated with sh-SC, sh16, or sh10 for 1 month (upper panels) or 3 months (lower panels) (n = 4-6 per group). Data are expressed as mean ± SEM, with statistical significance denoted by **P < 0.01, determined using an unpaired, two-tailed Student’s t-test. Scale bars represent 50 µm (A and C) and 20 µm (E and F).

Article Snippet: Sections were incubated overnight at 4 °C with primary antibodies against LDB3, filamin C, BAG3, HSPA8, and EGFP (Cell Signaling Technology, ab184601); antibody details and dilutions were as described previously.

Techniques: shRNA, Two Tailed Test