Identification of compounds that rescue otic and myelination defects in the zebrafish adgrg6 (gpr126) mutant

Elife. 2019 Jun 10:8:e44889. doi: 10.7554/eLife.44889.

Abstract

Adgrg6 (Gpr126) is an adhesion class G protein-coupled receptor with a conserved role in myelination of the peripheral nervous system. In the zebrafish, mutation of adgrg6 also results in defects in the inner ear: otic tissue fails to down-regulate versican gene expression and morphogenesis is disrupted. We have designed a whole-animal screen that tests for rescue of both up- and down-regulated gene expression in mutant embryos, together with analysis of weak and strong alleles. From a screen of 3120 structurally diverse compounds, we have identified 68 that reduce versican b expression in the adgrg6 mutant ear, 41 of which also restore myelin basic protein gene expression in Schwann cells of mutant embryos. Nineteen compounds unable to rescue a strong adgrg6 allele provide candidates for molecules that may interact directly with the Adgrg6 receptor. Our pipeline provides a powerful approach for identifying compounds that modulate GPCR activity, with potential impact for future drug design.

Keywords: Adgrg6 (Gpr126); adhesion GPCR; chemoinformatics; developmental biology; inner ear; myelination; neuroscience; phenotypic screening; zebrafish.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Ear, Inner / drug effects
  • Ear, Inner / embryology
  • Ear, Inner / metabolism*
  • Embryo, Nonmammalian / drug effects
  • Embryo, Nonmammalian / embryology
  • Embryo, Nonmammalian / metabolism
  • Gene Expression Regulation, Developmental / drug effects
  • Molecular Structure
  • Mutation
  • Myelin Sheath / drug effects
  • Myelin Sheath / metabolism*
  • Peripheral Nervous System / drug effects
  • Peripheral Nervous System / metabolism*
  • Proteoglycans / genetics
  • Proteoglycans / metabolism
  • Receptors, G-Protein-Coupled / genetics
  • Receptors, G-Protein-Coupled / metabolism*
  • Schwann Cells / drug effects
  • Schwann Cells / metabolism
  • Signal Transduction / drug effects
  • Signal Transduction / genetics
  • Small Molecule Libraries / chemistry
  • Small Molecule Libraries / pharmacology
  • Zebrafish
  • Zebrafish Proteins / genetics
  • Zebrafish Proteins / metabolism*

Substances

  • Proteoglycans
  • Receptors, G-Protein-Coupled
  • Small Molecule Libraries
  • Zebrafish Proteins
  • stx7l protein, zebrafish
  • vcanb protein, zebrafish