C57BL/6JCya-Gjb2em1flox/Cya
Common Name
Gjb2-flox
Product ID
S-CKO-02641
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-14619-Gjb2-B6J-VA
When using this mouse strain in a publication, please cite “Gjb2-flox Mouse (Catalog S-CKO-02641) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Gjb2-flox
Strain ID
CKOCMP-14619-Gjb2-B6J-VA
Gene Name
Product ID
S-CKO-02641
Gene Alias
Cx26, Cxne, Cnx26, Gjb-2
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 14
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000055698
NCBI RefSeq
NM_008125
Target Region
Exon 2
Size of Effective Region
~3.0 kb
Overview of Gene Research
Gjb2, also known as Gap junction protein beta-2, encodes the transmembrane protein connexin 26 (Cx26). Gap junctions formed by connexins play crucial roles in intercellular communication, allowing the exchange of small molecules and ions, which is essential for normal cellular function and tissue homeostasis [1].
Mutations in Gjb2 are the most common genetic cause of non-syndromic hereditary deafness in humans, especially the 35delG and 235delC mutations [2]. Through the construction of heterozygous Gjb2+/35delG and Gjb2+/235delC mutant mice, and a homozygous mutant mouse model Gjb235delG/35delG, it was demonstrated that Gjb2 35delG disrupts the function and formation of intercellular gap junction channels of the cochlea rather than affecting the survival and function of hair cells. These mouse models are ideal for understanding the pathogenic mechanism of DFNB1A-related hereditary deafness [2].
In conclusion, Gjb2 is essential for normal cochlear function through its role in forming intercellular gap junction channels. The Gjb2-related mouse models have significantly contributed to understanding the pathogenesis of Gjb2-associated hearing loss, providing a basis for developing potential treatment strategies for this disease.
References：
1. Posukh, Olga L, Maslova, Ekaterina A, Danilchenko, Valeriia Yu, Zytsar, Marina V, Orishchenko, Konstantin E. 2023. Functional Consequences of Pathogenic Variants of the GJB2 Gene (Cx26) Localized in Different Cx26 Domains. In Biomolecules, 13, . doi:10.3390/biom13101521. https://pubmed.ncbi.nlm.nih.gov/37892203/
2. Li, Qing, Cui, Chong, Liao, Rongyu, Li, Huawei, Shu, Yilai. 2023. The pathogenesis of common Gjb2 mutations associated with human hereditary deafness in mice. In Cellular and molecular life sciences : CMLS, 80, 148. doi:10.1007/s00018-023-04794-9. https://pubmed.ncbi.nlm.nih.gov/37178259/
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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