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Gli-3 (also known as Zinc Finger Protein Gli-3 or GLI-Kruppel family member GLI-3) belongs to the GLI C2H2-type zinc-finger protein family and contains 5 C2H2-type zinc fingers. Gli-3 is very important for normal limb and brain development and is implicated in the transduction of Shh signal. Gli-3 is a nuclear protein expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium. Defects in Gli-3 are the cause of Greig cephalo-polysyndactyly syndrome (GCPS); an autosomal dominant disorder-affecting limb and craniofacial development. Two isoforms of human Gli-3 have been reported. One is the fulllength protein at ~170-190kDa and the other is a truncated isoform at ~80kDa.
