Vascular defects of DYRK1A knockouts are ameliorated by modulating calcium signaling in zebrafish

Dis Model Mech. 2019 May 23;12(5):dmm037044. doi: 10.1242/dmm.037044.

Abstract

DYRK1A is a major causative gene in Down syndrome (DS). Reduced incidence of solid tumors such as neuroblastoma in DS patients and increased vascular anomalies in DS fetuses suggest a potential role of DYRK1A in angiogenic processes, but in vivo evidence is still scarce. Here, we used zebrafish dyrk1aa mutant embryos to understand DYRK1A function in cerebral vasculature formation. Zebrafish dyrk1aa mutants exhibited cerebral hemorrhage and defects in angiogenesis of central arteries in the developing hindbrain. Such phenotypes were rescued by wild-type dyrk1aa mRNA, but not by a kinase-dead form, indicating the importance of DYRK1A kinase activity. Chemical screening using a bioactive small molecule library identified a calcium chelator, EGTA, as one of the hits that most robustly rescued the hemorrhage. Vascular defects of mutants were also rescued by independent modulation of calcium signaling by FK506. Furthermore, the transcriptomic analyses supported the alterations of calcium signaling networks in dyrk1aa mutants. Together, our results suggest that DYRK1A plays an essential role in angiogenesis and in maintenance of the developing cerebral vasculature via regulation of calcium signaling, which may have therapeutic potential for DYRK1A-related vascular diseases.

Keywords: DYRK1A; Hemorrhage; Vascular development; Zebrafish embryo.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blood Vessels / drug effects
  • Blood Vessels / pathology*
  • Brain / blood supply
  • Brain / embryology
  • Brain / pathology
  • Brain / ultrastructure
  • Calcium Signaling*
  • Cerebral Hemorrhage / pathology
  • Egtazic Acid / pharmacology
  • Embryo, Nonmammalian / metabolism
  • Embryo, Nonmammalian / pathology
  • Embryonic Development / drug effects
  • Endothelial Cells / drug effects
  • Endothelial Cells / metabolism
  • Gene Knockout Techniques*
  • Harmine / pharmacology
  • Intracellular Space / metabolism
  • Mutation / genetics
  • Phenotype
  • Protein Kinases / metabolism*
  • Transcriptome / genetics
  • Zebrafish / embryology
  • Zebrafish / metabolism*
  • Zebrafish Proteins / antagonists & inhibitors
  • Zebrafish Proteins / metabolism*

Substances

  • Zebrafish Proteins
  • Harmine
  • Egtazic Acid
  • Protein Kinases
  • dyrk1aa protein, zebrafish