Zinc-finger proteins contain DNA-binding domains and have a wide variety of functions, most of which encompass some form of transcriptional activation or repression. The majority of zinc-finger proteins contain a Krppel-type DNA binding domain and a KRAB domain, which is thought to interact with KAP1, thereby recruiting histone modifying proteins. GLI-3 (GLI family zinc finger 3), also known as GLI3FL (GLI3 full length protein), PHS, ACLS, GCPS, PAPA, PAPB, PAPA1 or PPDIV, is a 1,580 amino acid nuclear and cytoplasmic protein that acts as both a transcriptional activator and a repressor of the Sonic hedgehog (Shh) pathway. A member of the GLI C2H2-type zinc-finger protein family, GLI-3 is encoded by a gene that maps to human chromosome 7p14.1. Defects in the GLI-3 gene are the cause of a disorder known as Greig cephalo-poly-syndactyly syndrome (GCPS), which affects limb and craniofacial development.
