C57BL/6JCya-Cdh23em1flox/Cya
Common Name
Cdh23-flox
Product ID
S-CKO-06571
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-22295-Cdh23-B6J-VA
When using this mouse strain in a publication, please cite “Cdh23-flox Mouse (Catalog S-CKO-06571) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Cdh23-flox
Strain ID
CKOCMP-22295-Cdh23-B6J-VA
Gene Name
Product ID
S-CKO-06571
Gene Alias
v, ahl, bob, bus, ahl1, mdfw, sals, USH1D, nmf112, nmf181, nmf252, 4930542A03Rik
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 10
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000105464
NCBI RefSeq
NM_001252635
Target Region
Exon 5
Size of Effective Region
~0.6 kb
Overview of Gene Research
Cdh23, encoding a calcium-dependent transmembrane glycoprotein, forms the upper part of tip-links in inner ear hair cells, which are crucial for mechano-electrical transduction (MET) [2]. The gene is composed of 69 exons, and its cytoplasmic tail interacts with other proteins like harmonin in the upper tip-link density (UTLD) [2]. This function is essential for the transformation of acoustical waves into neurological signals in the inner ear [4].
Genetically modified mutant mice lacking Cdh23 exon 68 have compromised tip-link stability, resulting in progressive and noise-induced hearing loss, indicating that exon 68 inclusion is critical for tip-link stability through regulating condensate formation of UTLD components [2]. In zebrafish, knockout of cdh23 gene leads to a significant decrease in the response to sound stimulation, and genes like atp1b2b and myof may affect hearing by regulating ATP production and purine metabolism in cooperation with cdh23 [3].
In conclusion, Cdh23 is vital for the stability of tip-links in inner ear hair cells and the mechano-electrical transduction process. Studies on gene-knockout models in mice and zebrafish have revealed its role in hearing-related biological processes. Mutations in Cdh23 are associated with a broad spectrum of hearing loss, from non-syndromic to syndromic, and from congenital to age-related, providing insights into the molecular mechanisms of hearing loss [1].
References：
1. Usami, Shin-Ichi, Isaka, Yuichi, Miyagawa, Maiko, Nishio, Shin-Ya. 2022. Variants in CDH23 cause a broad spectrum of hearing loss: from non-syndromic to syndromic hearing loss as well as from congenital to age-related hearing loss. In Human genetics, 141, 903-914. doi:10.1007/s00439-022-02431-2. https://pubmed.ncbi.nlm.nih.gov/35020051/
2. 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/
3. 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/
4. Abitbol, Marie, Jagannathan, Vidhya, Lopez, Marie, Gache, Vincent, Leeb, Tosso. 2022. A CDH23 missense variant in Beauceron dogs with non-syndromic deafness. In Animal genetics, 54, 73-77. doi:10.1111/age.13273. https://pubmed.ncbi.nlm.nih.gov/36308003/
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
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