PUBLICATION
Three-Dimensional Structure of Inner Ear Hair Cell Ribbon Synapses in a Zebrafish Model of Usher Syndrome Type 1B
- Authors
- Riley, K.C., Koleilat, A., Dugdale, J.A., Cooper, S.A., Christensen, T.A., Schimmenti, L.A.
- ID
- ZDB-PUB-230420-52
- Date
- 2023
- Source
- Zebrafish 20: 475447-54 (Journal)
- Registered Authors
- Schimmenti, Lisa A.
- Keywords
- hair cells, ribbon synapse, serial block-face scanning electron microscopy, zebrafish
- MeSH Terms
-
- Animals
- Hair
- Hair Cells, Auditory, Inner/metabolism
- Hair Cells, Auditory, Inner/ultrastructure
- Humans
- Myosins/genetics
- Myosins/metabolism
- Synapses/metabolism
- Synapses/ultrastructure
- Usher Syndromes*/genetics
- Usher Syndromes*/metabolism
- Zebrafish*
- Zebrafish Proteins/genetics
- Zebrafish Proteins/metabolism
- PubMed
- 37071854 Full text @ Zebrafish
Citation
Riley, K.C., Koleilat, A., Dugdale, J.A., Cooper, S.A., Christensen, T.A., Schimmenti, L.A. (2023) Three-Dimensional Structure of Inner Ear Hair Cell Ribbon Synapses in a Zebrafish Model of Usher Syndrome Type 1B. Zebrafish. 20:475447-54.
Abstract
Our understanding of inner ear hair cell ultrastructure has heretofore relied upon two-dimensional imaging; however, serial block-face scanning electron microscopy (SBFSEM) changes this paradigm allowing for three-dimensional evaluation. We compared inner ear hair cells of the apical cristae in myo7aa-/- null zebrafish, a model of human Usher Syndrome type 1B, to hair cells in wild-type zebrafish by SBFSEM to investigate possible ribbon synapse ultrastructural differences. Previously, it has been shown that compared to wild type, myo7aa-/- zebrafish neuromast hair cells have fewer ribbon synapses yet similar ribbon areas. We expect the recapitulation of these results within the inner ear apical crista hair cells furthering the knowledge of three-dimensional ribbon synapse structure while resolving the feasibility of therapeutically targeting myo7aa-/- mutant ribbons. In this report, we evaluated ribbon synapse number, volume, surface area, and sphericity. Localization of ribbons and their distance from the nearest innervation were also evaluated. We determined that myo7aa-/- mutant ribbon synapses are smaller in volume and surface area; however, all other measurements were not significantly different from wild-type zebrafish. Because the ribbon synapses are nearly indistinguishable between the myo7aa-/- mutant and wild type, it suggests that the ribbons are structurally receptive, supporting that therapeutic intervention may be feasible.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping