PUBLICATION

Otopetrin 1 is required for otolith formation in the zebrafish Danio rerio

Authors
Hughes, I., Blasiole, B., Huss, D., Warchol, M.E., Rath, N.P., Hurle, B., Ignatova, E., David Dickman, J., Thalmann, R., Levenson, R., and Ornitz, D.M.
ID
ZDB-PUB-041208-7
Date
2004
Source
Developmental Biology   276(2): 391-402 (Journal)
Registered Authors
Blasiole, Brian, Levenson, Robert
Keywords
none
MeSH Terms
  • Animals
  • Calcium Carbonate/metabolism
  • Gene Expression Regulation, Developmental
  • Gravitation
  • Humans
  • In Situ Hybridization
  • Membrane Proteins/genetics
  • Membrane Proteins/metabolism*
  • Mice
  • Microscopy, Electron, Scanning
  • Morphogenesis
  • Oligonucleotides/genetics
  • Oligonucleotides/metabolism
  • Otolithic Membrane/anatomy & histology
  • Otolithic Membrane/chemistry
  • Otolithic Membrane/embryology*
  • Otolithic Membrane/metabolism
  • Saccule and Utricle/anatomy & histology
  • Saccule and Utricle/embryology
  • Saccule and Utricle/ultrastructure
  • X-Ray Diffraction
  • Zebrafish/anatomy & histology
  • Zebrafish/embryology*
  • Zebrafish/genetics
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
PubMed
15581873 Full text @ Dev. Biol.
Abstract
Orientation with respect to gravity is essential for the survival of complex organisms. The gravity receptor is one of the phylogenetically oldest sensory systems, and special adaptations that enhance sensitivity to gravity are highly conserved. The fish inner ear contains three large extracellular biomineral particles, otoliths, which have evolved to transduce the force of gravity into neuronal signals. Mammalian ears contain thousands of small particles called otoconia that serve a similar function. Loss or displacement of these structures can be lethal for fish and is responsible for benign paroxysmal positional vertigo (BPPV) in humans. The distinct morphologies of otoconial particles and otoliths suggest divergent developmental mechanisms. Mutations in a novel gene Otopetrin 1 (Otop1), encoding multi-transmembrane domain protein, result in nonsyndromic otoconial agenesis and a severe balance disorder in mice. Here we show that the zebrafish, Danio rerio, contains a highly conserved gene, otop1, that is essential for otolith formation. Morpholino-mediated knockdown of zebrafish Otop1 leads to otolith agenesis without affecting the sensory epithelium or other structures within the inner ear. Despite lack of otoliths in early development, otolith formation partially recovers in some fish after 2 days. However, the otoliths are malformed, misplaced, lack an organic matrix, and often consist of inorganic calcite crystals. These studies demonstrate that Otop1 has an essential and conserved role in the timing of formation and the size and shape of the developing otolith.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping