Figure 5  Haploinsufficiency of RanBP2 Causes a Decrease in HKI Protein and ATP Levels
(A) Quantitative analysis of NPCs in dissociated hippocampal neurons of wild-type (+/+) and heterozygote (+/−) mice upon immunostaining with mAb414. No difference in the density of NPCs (3–4 NPC/μm2) at the nuclear envelope was found between RanBP2+/+ and RanBP2+/− mice.
(B) Immunoblots with anti-RanBP2/Nup153/Nup62 (mAb414), −HKI, −mHsp70, and −Cox11 antibodies of retinal (top panel) and hippocampal homogenates of +/+ and +/− mice. In comparison to RanBP2+/+, RanBP2+/− mice exhibit a reduction in the expression levels of RanBP2 and HKI but not of other proteins.
(C) Quantitative analysis of relative protein expression levels of RanBP2, Cox11, HKI, and mHsp70 in the hippocampus of RanBP2+/+ and RanBP2+/−mice. There is ~2- and 4-fold reduction of RanBP2 and HKI in heterozygote mice.
(D) The level of HKI is reduced in the brain but not in other non-neuronal tissues tested (muscle, spleen, and liver).
(E) The total ATP level is reduced in the CNS tissues (brain and retina) but not in non-neuronal tissues tested (e.g., spleen).