Fig. 4: Cmpk2 deficiency impairs mitochondrial function in mouse neurons.
From: Loss of function of CMPK2 causes mitochondria deficiency and brain calcification
a ddPCR-based analysis of mtDNA copy number between WT and Cmpk2-KO mouse cortex tissues. n = 7~8, three replicates. b Representative images of mitochondria in the primary cultured cortical neurons of WT and Cmpk2-KO mice with antibodies against the mitochondrial marker ATP synthase β chain (green) and the cytoskeleton protein MAP2 (red); DAPI (blue) was used for nuclear staining. Scale bar, 5 μm. c Western blot of COX IV and cytochrome C in the WT and Cmpk2-KO mouse primary cultured cortical neurons. d Quantification of the change in protein levels of COX IV and cytochrome C. n = 3, three replicates. e Measurement of ATP level of the WT and Cmpk2-KO mouse neurons. f, g The Pi level in the neuron (f), CSF and serum (g) in the WT and Cmpk2-KO mice. h Representative transmission electron micrograph of mitochondria morphology in the primary cultured cortical neurons of WT and Cmpk2-KO mice. i–k Statistical graphs of cristae coverage ratio of primary cultured cortex neurons in WT and Cmpk2-KO mice. CC cristae coverage. Error bars indicate means ± SEM. ns not significant. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001; Student’s t-test. Scale bars, 2 μm in h, each left panel and 0.2 μm in h, each right panel.
