Acts as transcriptional activator. Required in the earliest stages in both axial midline development and left-right (LR) asymmetry specification. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'. Interacts with KPNA1 and KPNA6. Interacts (via C2H2-type domains 3, 4 and 5) with GLI3; the interaction enhances its transcriptional activity (By similarity). Interacts (via the C2H2-type domains 3, 4 and 5) with MDFIC (via the C2H2-type domains 3, 4 and 5); the interaction reduces its transcriptional activity. Translocation to the nucleus requires KPNA1 or KPNA6 (By similarity). Localizes in the cytoplasm in presence of MDFIC overexpression. CNS. A high level expression is seen in the cerebellum. Expressed in the CNS, tailbud and somites. The C2H2-type 3, 4 and 5 zinc finger domains are necessary for transcription activation. Some mice exhibit embryonic and postnatal lethality. Viable mice show heart disease, disturbances of laterality, neural tube defects and vertebral and rib defects. Belongs to the GLI C2H2-type zinc-finger protein family.