Combined treatment restores survival motor neuron (SMN) nuclear body gems in fibroblasts of a spinal muscular atrophy (SMA) patient. (A) Immunocytochemical analysis of the nuclear SMN body gems in wild-type (WT) and SMA human dermal fibroblasts (HDFs) treated with L-ascorbic acid (L-AA), sodium vanadate (SV) or L-AA combined with SV. SMN was stained with an SMN antibody (red, indicated by arrows). 4',6-Diamidino-2-phenylindole (DAPI) was used for nuclei staining. Bar: 50 μm. (B) The percentage of nuclei with gems (left) and the number of gems per 100 nuclei (right) in WT and SMA cells after different treatments were evaluated by immunocytochemical analysis. The mean ± SEM was calculated. *P < 0.01, **P < 0.01, and ***P < 0.001, Student's t test. NS = not significant.
Liu et al. BMC Medicine 2013 11:38 doi:10.1186/1741-7015-11-38