ZICs
Members
| ZIC1 | ZIC2 | ZIC3 | ZIC4 |
ZICs are genes encoding putative zinc finger transcription factors identified in mouse. All of the Zic proteins (ZICs) contain 5 C2H2 zinc fingers within a domain that shows homology with the Drosophila pair-rule gene, ’odd paired’ (opa).
Among its functions, opa is required for the appropriate expression of ’wingless,’ a segment-polarity gene that interacts with ’hedgehog’ to maintain parasegmental identity.
Notably, SHH (sonic hedgehog) (MIM.600725) and a vertebrate homolog of ’wingless’ (MIM.164820) are implicated in left-right axis formation in chick.
Pathology
ZIC1: heterozygous deletion in Dandy-Walker malformation
ZIC2 mutations in sporadic holoprosencephaly type 5
ZIC3 mutations in
- X-linked visceral heterotaxy (MIM.306955)
- X-linked transposition of the great arteries
- non-heterotaxy congenital heart disease (atrial septal defect and pulmonic stenosis)
ZIC4: heterozygous deletion in Dandy-Walker malformation