Figure 3. V171L and Q191X destabilize the expression of GINS4 and other GINS proteins and impair GINS complex assembly.
(A) GINS4 and GINS1 protein expression in BLCL derived from siblings and parents compared with those from 2 healthy donors (HD). Relative GINS4 protein expression of family members and 2 HDs from 3 independent experiments. (B) GINS complex 3D structure showing the position of V171L and Q191X variants. (C) GINS2 and GINS3 protein expression in BLCL derived from siblings and parents compared with 2 HD. One representative experiment of 3 independent experiments is shown (D) HEK293T cells were transiently transfected with Myc-GINS4 WT, Myc-GINS4-V171L, or Myc-GINS4 Q191X plasmids. After 48 hours, whole cellular extracts were immunoprecipitated with anti-MYC, and blots were probed for GINS1, GINS2, GINS3, and MYC. Data are representative of 3 independent experiments.