C57BL/6JCya-Cdh23em1/Cya
Common Name:
Cdh23-KO
Product ID:
S-KO-18943
Background:
C57BL/6JCya
Product Type
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Genotype
Sex
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Basic Information
Strain Name
Cdh23-KO
Strain ID
KOCMP-22295-Cdh23-B6J-VB
Gene Name
Product ID
S-KO-18943
Gene Alias
4930542A03Rik; USH1D; ahl; ahl1; bob; bus; mdfw; nmf112; nmf181; nmf252; sals; v
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
10
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Cdh23em1/Cya mice (Catalog S-KO-18943) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000105464
NCBI RefSeq
NM_001252635
Target Region
Exon 5
Size of Effective Region
~1.0 kb
Detailed Document
Overview of Gene Research
Cdh23, encoding a calcium-dependent transmembrane glycoprotein, forms the upper part of tip-links in inner ear hair cells [1]. These tip-links are crucial for mechano-electrical transduction (MET) in hair cells, an essential process for converting acoustical waves into neurological signals [1,2].
Disrupting Cdh23 exon 68 splicing in mice leads to progressive and noise-induced hearing loss due to compromised tip-link stability, even though tip-link formation is not affected [1]. In zebrafish, knockout of cdh23 using Nuclease technology technology causes a significant decrease in the response to sound stimulation, and transcriptomic analysis shows that genes like atp1b2b and myof may affect hearing by regulating ATP production and purine metabolism in cooperation with cdh23 [2].
In conclusion, Cdh23 is vital for maintaining the stability of tip-links in inner ear hair cells, which is essential for normal hearing. Studies using gene-knockout models in mice and zebrafish have revealed its role in hearing-related biological processes, especially in understanding the mechanisms underlying hearing loss [1,2].
References：
1. Li, Nana, Liu, Shuang, Zhao, Dange, Xiong, Wei, Xu, Zhigang. 2024. Disruption of Cdh23 exon 68 splicing leads to progressive hearing loss in mice by affecting tip-link stability. In Proceedings of the National Academy of Sciences of the United States of America, 121, e2309656121. doi:10.1073/pnas.2309656121. https://pubmed.ncbi.nlm.nih.gov/38408254/
2. Yang, Shu, Xie, Bing-Lin, Dong, Xiao-Ping, Xiao, Zi-An, Xie, Ding-Hua. 2023. cdh23 affects congenital hearing loss through regulating purine metabolism. In Frontiers in molecular neuroscience, 16, 1079529. doi:10.3389/fnmol.2023.1079529. https://pubmed.ncbi.nlm.nih.gov/37575969/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen