The Axenfeld-Rieger Syndrome Gene FOXC1 Contributes to Left-Right Patterning.

ABSTRACT: Precise spatiotemporal expression of the Nodal-Lefty-Pitx2 cascade in the lateral plate mesoderm establishes the left-right axis, which provides vital cues for correct organ formation and function. Mutations of one cascade constituent PITX2 and, separately, the Forkhead transcription factor FOXC1 independently cause a multi-system disorder known as Axenfeld-Rieger syndrome (ARS). Since cardiac involvement is an established ARS phenotype and because disrupted left-right patterning can cause congenital heart defects, we investigated in zebrafish whether foxc1 contributes to organ laterality or situs. We demonstrate that CRISPR/Cas9-generated foxc1a and foxc1b mutants exhibit abnormal cardiac looping and that the prevalence of cardiac si

SUBMITTER: Chrystal PW 

PROVIDER: S-EPMC7912076 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature