Figure 4.

Knockdown of Ict1 in Control Fibroblasts Results in a Mitochondrial Translation Defect Similar to that in Patients with C12orf65 Mutations

(A and B) Control fibroblasts were transiently transfected with small interfering RNA constructs specific to Ict1 and analyzed by BN-PAGE (A) and [³⁵S] Met/Cys-labeling (B) of mitochondrial polypeptides.

(C) Control fibroblasts that were untreated, overexpressing, or depleted for Ict1 were analyzed by immunoblotting for the presence of mitochondrial ribosomal proteins (DAP3 and MRPL32). A patient with a mitochondrial ribosome assembly defect (Comb. def. patient) was used as a positive control.