Fig. 9
Alteration in FLNC aggregation in HSPB7 mutant CMs. (A) Filter trap assay probing for Filamin-C in lysates from day 10 hESC-derived cardiomyocytes treated with bafilomycin A shows increase in FLNC-containing aggregates in HPSB7-/- cardiomyocytes (lines 26 and 30) compared to wildtype (parental WT and non-targeted line 12). Shown is a representative image from multiple reproducible experiments. (B) Quantification of filter trap assays. Changes are significant according to an unpaired t-test, corrected for multiple comparisons with the Holm-Sidak method (p<0.0001). (C) Immunofluorescence for cardiac troponin (red) and FLNC (green) in hESC-CMs derived from HSPB7 wildtype or (D) HSPB7 mutant lines following treatment with bafilomycin A. White arrowheads mark representative abnormal puncta. |
Reprinted from Developmental Biology, 435(1), Mercer, E.J., Lin, Y.F., Cohen-Gould, L., Evans, T., Hspb7 is a Cardioprotective Chaperone Facilitating Sarcomeric Proteostasis, 41-55, Copyright (2018) with permission from Elsevier. Full text @ Dev. Biol.