Figure 7.
Validation of CHCHD2 Dysregulation and Mitochondrial Respiratory Deficits in CAG180 HD Cells
(A) CHCHD2 qRT-PCR in CAG180 and isogenic control hiPSCs; n = 3 per clone; values for three independent biological replicates shown as mean ± SEM; ns, no significance; and ∗∗∗p < 0.001 was determined by unpaired t test.
(B) CHCHD2 protein levels in CAG180 and isogenic control hiPSCs and NPCs; representative blot shown; n = 3 per clone; values for three independent biological replicates shown as mean ± SEM; ∗p < 0.05 was determined by unpaired t test.
(C–F) Measures of mitochondrial respiration in CAG180 and isogenic control NPCs. (C) Mitochondrial oxidative phosphorylation; (D) mitochondrial basal respiration; (E) mitochondrial ATP production; and (F) mitochondrial maximal respiration; for (D) (E), and (F) n = 5 independent biological replicates; values shown as mean ± SEM; ns, no significance; ∗p < 0.05 and ∗∗p < 0.01, was determined by paired t test.