Figure 5. Transfection of mutant FHL1 H123Y and C132F in C2C12 cells.
(A) C2C12 myotubes transfected with HA-tagged FHL1 mutant H123Y. Arrows indicate inclusions clustered around a nucleus (blue). (B) Quantification of inclusion-carrying cells as percentage of transfected cells. n = 3; 100 cells counted each; error bars represent SEM. *P < 0.05. (C–F) Inclusions (arrows) in differentiated C2C12 cells induced by transfection of mutant FHL1 H123Y-HA or C132F-HA (data not shown) recapitulate findings seen in muscle biopsy sections of patients by including proteins typically associated with aggresomes, including ubiquitin (C) and the endoplasmic reticulum chaperone GRP78 (BiP) (D) as well as other endogenous muscle proteins such as the intermediate filament desmin (E) and the lysosome-associated protein LAMP1 (F). Scale bars: 10 μm.