Zinc finger protein ZIC 3 (ZIC3)

Zinc finger protein ZIC 3 (ZIC3) is a member of the ZIC protein family, defined by five conserved C2H2 zinc finger domains that mediate DNA binding and protein interactions[1][2][3][4][5]. It acts as a transcription factor, playing essential roles in early embryonic development, particularly the establishment of left-right body axis, axial midline formation, and maintenance of embryonic stem cell pluripotency[1][3][5]. ZIC3 can inhibit Wnt signaling via its zinc finger domains, contributing to tissue specification such as neural crest development[5]. Mutations in ZIC3 disrupt its nuclear localization and transcriptional activity, causing congenital defects including heterotaxy (abnormal left-right organ arrangement), heart malformations, neural tube defects, and skeletal patterning abnormalities[1][2][3][4][5]. ZIC3 is also crucial for proper mesoderm and neural tissue expression, regulated by enhancers binding developmental transcription factors including Nanog, Oct4, Brachyury, Eomes, and LEF1[3]. There is no evidence ZIC3 is currently a direct therapeutic drug target; its main importance is as a developmental regulator and candidate gene for congenital disease research.

Other names

ZNF203HTXHTX1VACTERLXZinc finger protein 203Zic family member 3 (odd-paired homolog, Drosophila)heterotaxy 1zinc finger protein of the cerebellum 3