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ZFIN ID: ZDB-PUB-181006-2
Cog4 is required for protrusion and extension of the epithelium in the developing semicircular canals
Clément, A., Blanco-Sánchez, B., Peirce, J.L., Westerfield, M.
Date: 2018
Source: Mechanisms of Development   155: 1-7 (Journal)
Registered Authors: Blanco, Bernardo, Clément, Aurélie, Peirce, Judy, Westerfield, Monte
Keywords: Cog4, Inner ear, Pillar, Semicircular canal, Zebrafish
MeSH Terms:
  • Animals
  • Epithelium/metabolism*
  • Extracellular Matrix/metabolism
  • Gene Expression Regulation, Developmental/physiology
  • Hair Cells, Auditory, Inner/metabolism
  • Hearing Loss/metabolism
  • Morphogenesis/physiology
  • Mutation/physiology
  • Semicircular Canals/growth & development*
  • Semicircular Canals/metabolism*
  • Zebrafish/growth & development
  • Zebrafish/metabolism
  • Zebrafish Proteins/metabolism*
PubMed: 30287385 Full text @ Mech. Dev.
The semicircular canals in the inner ear sense angular acceleration. In zebrafish, the semicircular canals develop from epithelial projections that grow toward each other and fuse to form pillars. The growth of the epithelial projections is driven by the production and secretion of extracellular matrix components by the epithelium. The conserved oligomeric Golgi 4 protein, Cog4, functions in retrograde vesicle transport within the Golgi and mutations can lead to sensory neural hearing loss. In zebrafish cog4 mutants, the inner ear is smaller and the number of hair cells is reduced. Here, we show that formation of the pillars is delayed and that secretion of extracellular matrix components (ECM) is impaired in cog4-/- mutants. These results show that Cog4 is required for secretion of ECM molecules essential to drive the growth of the epithelial projections and thus regulates morphogenesis of the semicircular canals.