PUBLICATION
MicroRNAs in hair cell development and deafness
- Authors
- Li, H., and Fekete, D.M.
- ID
- ZDB-PUB-100820-27
- Date
- 2010
- Source
- Current opinion in otolaryngology & head and neck surgery 18(5): 459-465 (Review)
- Registered Authors
- Fekete, Donna Marie
- Keywords
- none
- MeSH Terms
-
- Animals
- Deafness/genetics
- Deafness/physiopathology*
- Hair Cells, Auditory/physiology*
- Humans
- Labyrinth Diseases/genetics
- Labyrinth Diseases/physiopathology*
- MicroRNAs/genetics*
- Mutation
- PubMed
- 20717030 Full text @ Curr. Opin. Otolaryngol. Head Neck Surg.
Citation
Li, H., and Fekete, D.M. (2010) MicroRNAs in hair cell development and deafness. Current opinion in otolaryngology & head and neck surgery. 18(5):459-465.
Abstract
PURPOSE OF REVIEW: The identification of transcriptional activators and repressors of hair cell fates has recently been augmented by the discovery of microRNAs (miRNAs) that can function as post-transcriptional repressors in sensory hair cells.
RECENT FINDINGS: miRNAs are approximately 21-nucleotide single-stranded ribonucleic acids that can each repress protein synthesis of many target genes by interacting with messenger RNA transcripts. A triplet of these miRNAs, the miR-183 family, is highly expressed in vertebrate hair cells, as well as a variety of other peripheral neurosensory cells. Point mutations in one member of this family, miR-96, underlie DFNA50 autosomal deafness in humans and lead to abnormal hair cell development and survival in mice. In zebrafish, overexpression of the miR-183 family induces extra and ectopic hair cells, whereas knockdown reduces hair cell numbers. Genetically engineered mice with a block in miRNA biosynthesis during early ear development, or during hair cell differentiation, reveal the necessity of miRNAs at these crucial time points.
SUMMARY: Because miRNAs can simultaneously down-regulate dozens to perhaps hundreds of transcripts, they will soon be explored as potential therapeutic agents to repair or regenerate hair cells in animal models.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping