PUBLICATION
The role of cilia in the development, survival, and regeneration of hair cells
- Authors
- Boldizar, H., Friedman, A., Stanley, T., Padilla, M., Galdieri, J., Sclar, A., Stawicki, T.M.
- ID
- ZDB-PUB-240912-17
- Date
- 2024
- Source
- Biology Open 13(9): (Journal)
- Registered Authors
- Stawicki, Tamara
- Keywords
- Cilia, Hair Cells, Intraflagellar Transport, Mitochondria
- MeSH Terms
-
- Animals
- Apoptosis/genetics
- Cell Proliferation
- Cell Survival*/genetics
- Cilia*/metabolism
- Hair Cells, Auditory/metabolism
- Hair Cells, Auditory/physiology
- Membrane Potential, Mitochondrial
- Mitochondria/genetics
- Mitochondria/metabolism
- Mutation*
- Regeneration*
- Zebrafish*
- Zebrafish Proteins*/genetics
- Zebrafish Proteins*/metabolism
- PubMed
- 39263863 Full text @ Biol. Open
Citation
Boldizar, H., Friedman, A., Stanley, T., Padilla, M., Galdieri, J., Sclar, A., Stawicki, T.M. (2024) The role of cilia in the development, survival, and regeneration of hair cells. Biology Open. 13(9):.
Abstract
Mutations impacting cilia genes lead to a class of human diseases known as ciliopathies. This is due to the role of cilia in the development, survival, and regeneration of many cell types. We investigated the extent to which disrupting cilia impacted these processes in lateral line hair cells of zebrafish. We found that mutations in two intraflagellar transport (IFT) genes, ift88 and dync2h1, which lead to the loss of kinocilia, caused increased hair cell apoptosis. IFT gene mutants also have a decreased mitochondrial membrane potential, and blocking the mitochondrial uniporter causes a loss of hair cells in wild-type zebrafish but not mutants, suggesting mitochondria dysfunction may contribute to the apoptosis seen in these mutants. These mutants also showed decreased proliferation during hair cell regeneration but did not show consistent changes in support cell number or proliferation during hair cell development. These results show that the loss of hair cells seen following disruption of cilia through either mutations in anterograde or retrograde IFT genes appears to be due to impacts on hair cell survival but not necessarily development in the zebrafish lateral line.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping