Increased cofilin-1 expression alters actin dynamics in Emery-Dreifuss muscular dystrophy (EDMD)
(A) Schematic representation of actin dynamics mechanisms.
(B) Immunoblots showing pERK1/2, ERK1/2, cofilin-1, profilin-1, ARP2, and N-WASP protein level in soleus from old WT (n = 5) and Lmnap.H222P/H222P (H222P) (n = 5) mice. GAPDH is shown as loading control.
(C) Quantification of pERK1/2, ERK1/2, cofilin-1, profilin-1, ARP2, and N-WASP protein expression level in soleus from old WT (n = 5) and Lmnap.H222P/H222P (H222P) (n = 5) mice. ∗∗p ≤ 0.001 between old WT and Lmnap.H222P/H222P (H222P). Data are represented as mean ± SD.
(D) Immunoblots showing cofilin-1protein level in skeletal muscle from EDMD patient carrying LMNA mutation. GAPDH is shown as loading control. Data are represented as mean ± SD.
(E) Immunoblot showing G-actin and F-actin protein levels in soleus from old WT (n = 3) and Lmnap.H222P/H222P (H222P) (n = 3) mice.