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Dev Biol 2012 Apr 01;3641:22-31. doi: 10.1016/j.ydbio.2012.01.011.
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An essential and highly conserved role for Zic3 in left-right patterning, gastrulation and convergent extension morphogenesis.

Cast AE , Gao C , Amack JD , Ware SM .

Mutations in ZIC3 result in X-linked heterotaxy in humans, a syndrome consisting of left-right (L-R) patterning defects, midline abnormalities, and cardiac malformations. Similarly, loss of function of Zic3 in mouse results in abnormal L-R patterning and cardiac development. However, Zic3 null mice also exhibit defects in gastrulation, neural tube closure, and axial patterning, suggesting the hypothesis that Zic3 is necessary for proper convergent extension (C-E) morphogenesis. To further investigate the role of Zic3 in early embryonic development, we utilized two model systems, Xenopus laevis and zebrafish, and performed loss of function analysis using antisense morpholino-mediated gene knockdown. Both Xenopus and zebrafish demonstrated significant impairment of C-E in Zic3 morphants. L-R patterning was also disrupted, indicating that the role of Zic3 in L-R axis development is conserved across species. Correlation of L-R patterning and C-E defects in Xenopus suggests that early C-E defects may underlie L-R patterning defects at later stages, since Zic3 morphants with moderate to severe C-E defects exhibited an increase in laterality defects. Taken together, these results demonstrate a functional conservation of Zic3 in L-R patterning and uncover a previously unrecognized role for Zic3 in C-E morphogenesis during early vertebrate development.

PubMed ID: 22285814
PMC ID: PMC3294024
Article link: Dev Biol
Grant support: [+]

Species referenced: Xenopus laevis
Genes referenced: ctrl nodal1 odc1 pitx2 tbxt tnni3 zic3
GO keywords: heart development [+]
Morpholinos: zic3 MO2 zic3 MO3 zic3 MO4

Disease Ontology terms: visceral heterotaxy
Phenotypes: Xla Wt + zic3 MO (fig.1.d, e) [+]

Article Images: [+] show captions
References [+] :
Antic, Planar cell polarity enables posterior localization of nodal cilia and left-right axis determination during mouse and Xenopus embryogenesis. 2010, Pubmed, Xenbase