Fig. 2: Histological examination of peripheral tissues from P361R-SMA mice.
From: Spinal muscular atrophy-like phenotype in a mouse model of acid ceramidase deficiency
Sections from spleens (a), livers (b) and bladders (c) of wild-type (WT) and P361R-SMA mice were stained using hematoxylin and eosin (H&E), or immunostained for macrophages (Mac-2) or lysosomes (Lamp1). Scale bars represent 50 µm in (a) and (b), and 100 µm in (c). WT and P361R-SMA liver homogenates were Western blotted, and densitometry was used to quantify Cathepsin D (d), cleaved Caspase 3 (e), total STAT3 (f), phosphorylated STAT3 (g), total NF-κB (h), and phosphorylated NF-κB (i) from the representative immunoblots. j Relative ratios are plotted, obtained by deriving band densities for the major expected band and normalized first to β-Actin, then to average density in WT. Bars represent mean values (n = 4 mice per genotype), error bars depict standard error of the mean, and points show measurements for individual mice where triangles are females and circles are males. Data were compared using Welch’s t test, **p < 0.005, *p < 0.05.
