C57BL/6JCya-Wfs1em1flox/Cya
Common Name:
Wfs1-flox
Product ID:
S-CKO-06685
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Wfs1-flox
Strain ID
CKOCMP-22393-Wfs1-B6J-VA
Gene Name
Product ID
S-CKO-06685
Gene Alias
wolframin
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
5
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Wfs1em1flox/Cya mice (Catalog S-CKO-06685) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000043964
NCBI RefSeq
NM_011716
Target Region
Exon 3
Size of Effective Region
~0.6 kb
Detailed Document
Overview of Gene Research
Wfs1, encoding a transmembrane structural protein wolframin, is essential for several biological processes. It is involved in proper inner ear function, and mutations in this gene are associated with Wolfram syndrome (WFS) and related disorders [2,3]. WFS is a rare, autosomal, recessive neurogenetic disorder affecting multiple organ systems, often characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness [2]. Understanding its function through genetic models is crucial for finding treatments.
In a transgenic mouse model (DISC1-N), single-nucleus RNA sequencing and other techniques uncovered a group of glutamatergic neurons in the basolateral amygdala (BLA) marked by Wfs1 expression [1]. These neurons had diminished firing ability and impaired communication with adjacent astrocytes in DISC1-N mice. Optogenetic activation of astrocytes could reinstate neuronal excitability, improving risk-assessment behavior. In Wolfram syndrome mouse models, depletion of Wfs1 compromises mitochondrial function, and restoring Wfs1 levels can improve mitochondrial functionality [4].
In conclusion, Wfs1 plays a key role in various biological processes such as neuronal function in the BLA and mitochondrial function. Studies using mouse models, like the DISC1-N and those with Wfs1 depletion, have revealed its importance in risk-assessment behavior and mitochondrial-related disease conditions associated with Wolfram syndrome. These models are valuable for understanding the mechanisms underlying Wfs1-related disorders and for developing potential therapeutic strategies.
References:
1. Zhou, Xinyi, Xiao, Qian, Liu, Yaohui, Yang, Fan, Tu, Jie. 2024. Astrocyte-mediated regulation of BLAWFS1 neurons alleviates risk-assessment deficits in DISC1-N mice. In Neuron, 112, 2197-2217.e7. doi:10.1016/j.neuron.2024.03.028. https://pubmed.ncbi.nlm.nih.gov/38642554/
2. Kõks, Sulev. 2023. Genomics of Wolfram Syndrome 1 (WFS1). In Biomolecules, 13, . doi:10.3390/biom13091346. https://pubmed.ncbi.nlm.nih.gov/37759745/
3. Lim, Hui Dong, Lee, So Min, Yun, Ye Jin, Oh, Seung-Ha, Lee, Sang-Yeon. 2023. WFS1 autosomal dominant variants linked with hearing loss: update on structural analysis and cochlear implant outcome. In BMC medical genomics, 16, 79. doi:10.1186/s12920-023-01506-x. https://pubmed.ncbi.nlm.nih.gov/37041640/
4. Zatyka, Malgorzata, Rosenstock, Tatiana R, Sun, Congxin, Barrett, Timothy, Sarkar, Sovan. . Depletion of WFS1 compromises mitochondrial function in hiPSC-derived neuronal models of Wolfram syndrome. In Stem cell reports, 18, 1090-1106. doi:10.1016/j.stemcr.2023.04.002. https://pubmed.ncbi.nlm.nih.gov/37163979/
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