Establishment of iPSC lines and zebrafish with loss-of-function AHDC1 variants: Models for Xia-Gibbs syndrome

•We described the establishment of the first biological models for Xia-Gibbs syndrome (XGS) study: three induced pluripotent stem cell (iPSC) lines derived from cells of XGS patients and a zebrafish strain with ahdc1 LoF variant obtained through CRISPR/Cas9-mediated editing.•The iPSC lines showed expression of pluripotency factors and have the capacity to differentiate into the three germ layers.•The zebrafish line is fertile and breeding between heterozygous and wild-type animals generated offspring in a genotypic proportion in agreement with Mendelian law.•These models may be relevant to investigate the gibbin protein functions, its role in neurodevelopment and XGS pathophysiological mechanisms. In addition, the detailed description of the methodology to establish these models will be of value to the community interested in genetic disease modeling.