Early activation of antioxidant response genes in <i>Npc1</i>^-/- cerebellum.

<p>A – E) Cerebellar RNA was prepared from 3-, 5-, and 7-week old wildtype (WT) and <i>Npc1</i>^-/- mice. Target gene mRNA levels were analyzed by qPCR using primers against glial fibrillary acidic protein (<i>Gfap</i>), hypoxia-inducible factor 1alpha (<i>Hif1a</i>), nuclear factor erythroid 2-related factor 2 (<i>Nfe2l2</i>), heme oxygenase 1 (<i>Hmox1</i>), and superoxide dismutase 1 (<i>Sod1</i>), standardized to <i>Ppia</i>. F) Immunoblot analysis of tissue homogenates of cerebellum from 3-week old WT and <i>Npc1</i>^-/- mice using antibodies against superoxide dismutase (MnSOD) and actin as a loading control. Bar graphs show the ratio of band intensities of MnSOD to actin expressed as percent of the average of WT on the same membrane. G) Immunoblot analysis of tissue homogenates of cerebellum from 3- or 5-week old WT and <i>Npc1</i>^-/- mice using antibodies against phospho-JNK and JNK and actin as a loading control. Bar graphs show the ratio of band intensities of phosphorylated to total JNK, and the ratio of JNK to actin expressed as percent of the average of WT on the same membrane. All bar graphs show the mean ± SEM. * p<0.05, <i>Npc1</i>^-/- vs. WT.