Figure 3. Cerebellar histology.
Representative histologic sections of the cerebellum stained with calbindin. (A) A 49-day-old Npc1+/+ wild-type mouse. (B) A 49-day-old Npc1−/− mouse with no treatment, demonstrating significant loss of Purkinje cells. (C) A 49-day-old Npc1−/− mouse treated with weekly injections of 2HPBCD starting at 7 days of age, demonstrating partial preservation of Purkinje cells. (D) A 49-day-old Npc1−/− mouse treated with continuous ICV infusion of 2HPBCD for 4 weeks starting at 21 days of age, with concomitant weekly injections of 2HPBCD starting at 7 days of age, demonstrating complete preservation of Purkinje cells.
2HPBCD: 2-hydroxypropyl-β-cyclodextrin; ICV: Intracerebroventricular; sc.: Subcutaneous.