GLIS2 is a 524 amino acid protein that belongs to the GLI C2H2-type zinc-finger protein family. By recruiting the corepressors CtBP1 and HDAC3, GLIS2 represses the transcriptional activation mediated by ∫-catenin in the Wnt pathway. GLIS2 can act either as a transcription repressor or as a transcription activator and may be involved in neuron differentiation. Mutations of GLIS2 may be associated with development of progressive chronic kidney disease with characteristics resembling nephronophthisis. GLIS2 contains five tandem Cys(2)-His(2) zinc finger motifs that exhibit the highest homology to those of members of the GLI and Zic subfamilies of Krüppel-like proteins. GLIS2 is expressed at high levels in kidney and at low levels in heart, lung and placenta.