zinc finger protein ZIC3; zinc finger protein 203; zinc finger protein of the cerebellum 3 (ZIC3, ZNF203)

Function

• acts as transcriptional activator
• required in the earliest stages in both axial midline development & left-right asymmetry specification
• binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'
• interacts (via the C2H2-type zinc finger domains 3, 4 & 5) with MDFIC
  • interaction reduces its transcriptional activity (putative)
• interacts with KPNA1 & KPNA6
• interacts (via C2H2-type zinc finger domains 3, 4 & 5) with GLI3;
  • interaction enhances its transcriptional activity

Structure

• the C2H2-type Zn+2 finger domains 3, 4 & 5 are necessary for transcription activation (putative)
• belongs to the GLI C2H2-type Zn+2-finger protein family
• contains 5 C2H2-type Zn+2 fingers

Compartment

• nucleus. cytoplasm (putative)
• localizes in the cytoplasm withf MDFIC overexpression (putative)
• translocation to the nucleus requires KPNA1 or KPNA6

Pathology

• defects in ZIC3 are the cause of visceral heterotaxy X-linked type 1
• defects in ZIC3 are a cause of VACTERL association X-linked with or without hydrocephalus

More general terms

• zinc finger protein of the cerebellum (ZIC)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7547
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7547
• OMIM: https://mirror.omim.org/entry/300265
• OMIM: https://mirror.omim.org/entry/306955
• OMIM: https://mirror.omim.org/entry/314390
• UniProt: http://www.uniprot.org/uniprot/O60481.html