Figure 5

Complement deficiency does not affect patterned Purkinje neuron death and disease progression. (A) Cerebellar PNs (anti-D28K, red) in the anterior zone (AZ) die before PNs in the central and posterior zone (CZ and PZ), and PNs in the nodular zone (NZ) survive regardless of age. This patterned PN loss was not altered by C1q deficiency. (B) C1q deficiency also did not change the level of activity of microglia cells, marked by anti-CD68 and quantified by percent area occupied in the tissue, throughout the brain. The cerebellar cortex of lobule III was used to determine the level of microglia activity at P60. (D) Patterned PN (anti-D28K, blue) loss and the inverse pattern of microglial (anti-CD68, yellow) activity was also detected in C3 ^-/-; Npc1 ^-/- mice. (C, E) Two-way ANOVA analysis on the weight profiles of Npc1 ^-/- mice showed that neither C1qa (red lines, P = 0.70) nor C3 (blue lines, P = 0.41) deficiency significantly affected disease progression. Non-C1qa or C3 deficient Npc1 ^-/- sibling mice were used for comparison (dashed lines).