5.5.2. The GACI Zebrafish

Similar to PXE and ABCC6, a zebrafish model of GACI was generated targeting the *Enpp1* gene [123]. Unlike the *abcc6a* KO counterparts, these mutant zebrafish developed ectopic calcifications similar to that seen in GACI and PXE models in a variety of soft tissues that included the skin, cartilage, heart, intracranial space, and the notochord sheet. Treatment with the bisphosphonate etidronate (a PPi analog) rescues some aspects of the phenotype. The report also noted that the expression of *Enpp1* in blood vessels or the floor plate of mutant embryos was able to rescue the notochord mineralization phenotype.
