The neurodevelopmental disorder risk gene DYRK1A is required for ciliogenesis and control of brain size in Xenopus embryos

Development. 2020 Jun 22;147(21):dev189290. doi: 10.1242/dev.189290.

Abstract

DYRK1A [dual specificity tyrosine-(Y)-phosphorylation-regulated kinase 1 A] is a high-confidence autism risk gene that encodes a conserved kinase. In addition to autism, individuals with putative loss-of-function variants in DYRK1A exhibit microcephaly, intellectual disability, developmental delay and/or congenital anomalies of the kidney and urinary tract. DYRK1A is also located within the critical region for Down syndrome; therefore, understanding the role of DYRK1A in brain development is crucial for understanding the pathobiology of multiple developmental disorders. To characterize the function of this gene, we used the diploid frog Xenopus tropicalis We discover that Dyrk1a is expressed in ciliated tissues, localizes to ciliary axonemes and basal bodies, and is required for ciliogenesis. We also demonstrate that Dyrk1a localizes to mitotic spindles and that its inhibition leads to decreased forebrain size, abnormal cell cycle progression and cell death during brain development. These findings provide hypotheses about potential mechanisms of pathobiology and underscore the utility of X. tropicalis as a model system for understanding neurodevelopmental disorders.

Keywords: Autism; Ciliogenesis; DYRK1A; Down Syndrome; Microtubules; Spindle.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / anatomy & histology*
  • Brain / embryology
  • Cell Cycle / genetics
  • Cell Survival
  • Cilia / metabolism*
  • Embryo, Nonmammalian / anatomy & histology*
  • Gene Expression Regulation, Developmental
  • Genetic Predisposition to Disease
  • Neurodevelopmental Disorders / genetics*
  • Organ Size
  • Organogenesis / genetics*
  • Phenotype
  • Protein Serine-Threonine Kinases / genetics*
  • Protein Serine-Threonine Kinases / metabolism
  • Protein-Tyrosine Kinases / genetics*
  • Protein-Tyrosine Kinases / metabolism
  • Risk Factors
  • Spindle Apparatus / metabolism
  • Telencephalon / anatomy & histology
  • Xenopus / embryology*
  • Xenopus / genetics*
  • Xenopus Proteins / genetics*
  • Xenopus Proteins / metabolism

Substances

  • Xenopus Proteins
  • Dyrk1A protein, Xenopus
  • Protein-Tyrosine Kinases
  • Protein Serine-Threonine Kinases