Logo
Homepage
Explore Our Models
My Cart
Contact
Subscribe
Models
Genetically Engineered Animals
Knockout Mice
Knockout Rats
Knockin Mice
Knockin Rats
Transgenic Mice
Transgenic Rats
Model Generation Techniques
Turboknockout<sup>®</sup> Gene Targeting
ES Cell Gene Targeting
Targeted Gene Editing
Regular Transgenic
PiggyBac Transgenesis
BAC Transgenic
Research Models
HUGO-GT™ Humanized Mice
Cre Mouse Lines
Humanized Target Gene Models
Metabolic Disease Models
Ophthalmic Disease Models
Neurological Disease Models
Autoimmune Disease Models
Immunodeficient Mouse Models
Humanized Immune System Mouse Models
Oncology & Immuno-oncology Models
Covid-19 Mouse Models
MouseAtlas Model Library
Knockout Cell Line Product Catalog
Tumor Cell Line Product Catalog
AAV Standard Product Catalog
Animal Supporting Services
Breeding Services
Cryopreservation & Recovery
Phenotyping Services
BAC Modification
Custom Cell Line Models
Induced Pluripotent Stem Cells (iPSCs)
Knockout Cell Lines
Knockin Cell Lines
Point Mutation Cell Lines
Overexpression Cell Lines
Virus Packaging
Adeno-associated Virus (AAV) Packaging
Lentivirus Packaging
Adenovirus Packaging
CRO Services
By Therapeutic Area
Oncology
Ophthalmology
Neuroscience
Metabolic & Cardiovascular Diseases
Autoimmune & Inflammatory
By Drug Type
AI-Powered AAV Discovery
Gene Therapy
Oligonucleotide Therapy
Antibody Therapy
Cell Immunotherapy
Resources
Promotion
Events & Webinars
Newsroom
Blogs & Insights
Resource Vault
Reference Databases
Peer-Reviewed Citations
Rare Disease Data Center
AbSeek
Cell iGeneEditor™ System
OriCell
Quality
Facility Overview
Animal Health & Welfare
Health Reports
About Us
Corporate Overview
Our Partners
Careers
Contact Us
Login
Request a Product Quote
Select products from our catalogs and submit your request. Our team will get back to you with detailed information.
Full Name
Email
Phone Number
Organization
Job Role
Country
Catalog Type
Product Name
Additional Comments
Cyagen values your privacy. We’d like to keep you informed about our latest offerings and insights. Your preferences:
You may unsubscribe from these communications at any time. See our Privacy Policy for details on opting out and data protection.
By clicking the button below, you consent to allow Cyagen to store and process the personal information submitted in this form to provide you the content requested.
C57BL/6JCya-Pjvkem1flox/Cya
Common Name:
Pjvk-flox
Product ID:
S-CKO-17737
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Pjvk-flox
Strain ID
CKOCMP-381375-Pjvk-B6J-VC
Gene Name
Pjvk
Product ID
S-CKO-17737
Gene Alias
Dfnb59; Gm1001
Background
C57BL/6JCya
NCBI ID
381375
Modification
Conditional knockout
Chromosome
2
Phenotype
MGI:2685847
Document
Click here to download >>
Application
--
More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pjvkem1flox/Cya mice (Catalog S-CKO-17737) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000099986
NCBI RefSeq
NM_001080711
Target Region
Exon 2~3
Size of Effective Region
~1.9 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Pjvk, also known as DFNB59 and encoding Pejvakin, is a gene within the gasdermin family [3,4,7]. Pejvakin is expressed in sensory hair cells and first-order neurons of the inner ear and is involved in generating auditory and neural signals in the inner ear [2,6]. Mutations in Pjvk cause autosomal recessive non-syndromic hearing impairment (AR-NSHI) of the DFNB59 type, and some cases may also show auditory neuropathy spectrum disorder (ANSD) [1].

In Pjvk -mutant mice, delivery of the PJVK transgene using the synthetic Anc80L65 vector led to robust exogenous pejvakin expression in hair cells and neurons of the cochlea and vestibular organs. This resulted in significant restoration of spiral ganglion neuron density and hair cells in the cochlea, partial recovery of sensorineural hearing impairment, and recovery of vestibular ganglion neurons and balance function to wild-type levels [2]. A knockin mouse model carrying the c.874G > A (p.G292R) variant was generated to study the auditory and vestibular phenotypes of DFNB59, as previous mouse models with different Pjvk defects showed phenotypic variability and inconsistency [5].

In conclusion, Pjvk plays a crucial role in inner-ear function, especially in auditory and vestibular systems. The study of Pjvk -mutant mouse models provides insights into the gene's function in these systems and offers potential for gene-therapy-based solutions for PJVK-related inner-ear deficits, specifically in treating hearing and balance disorders associated with Pjvk mutations [2,5].

References:
1. Domínguez-Ruiz, María, Rodríguez-Ballesteros, Montserrat, Gandía, Marta, Santarelli, Rosamaria, Del Castillo, Ignacio. 2022. Novel Pathogenic Variants in PJVK, the Gene Encoding Pejvakin, in Subjects with Autosomal Recessive Non-Syndromic Hearing Impairment and Auditory Neuropathy Spectrum Disorder. In Genes, 13, . doi:10.3390/genes13010149. https://pubmed.ncbi.nlm.nih.gov/35052489/
2. Lu, Ying-Chang, Tsai, Yi-Hsiu, Chan, Yen-Huei, Wu, Chen-Chi, Cheng, Yen-Fu. 2022. Gene therapy with a synthetic adeno-associated viral vector improves audiovestibular phenotypes in Pjvk-mutant mice. In JCI insight, 7, . doi:10.1172/jci.insight.152941. https://pubmed.ncbi.nlm.nih.gov/36278489/
3. De Schutter, Elke, Roelandt, Ria, Riquet, Franck B, Wullaert, Andy, Vandenabeele, Peter. 2021. Punching Holes in Cellular Membranes: Biology and Evolution of Gasdermins. In Trends in cell biology, 31, 500-513. doi:10.1016/j.tcb.2021.03.004. https://pubmed.ncbi.nlm.nih.gov/33771452/
4. Ouyang, Xiaohu, Zhou, Junfen, Lin, Lan, Luo, Shanshan, Hu, Desheng. 2023. Pyroptosis, inflammasome, and gasdermins in tumor immunity. In Innate immunity, 29, 3-13. doi:10.1177/17534259221143216. https://pubmed.ncbi.nlm.nih.gov/36632024/
5. Cheng, Yen-Fu, Tsai, Yi-Hsiu, Huang, Chun-Ying, Hsu, Chuan-Jen, Wu, Chen-Chi. 2020. Generation and pathological characterization of a transgenic mouse model carrying a missense PJVK mutation. In Biochemical and biophysical research communications, 532, 675-681. doi:10.1016/j.bbrc.2020.07.101. https://pubmed.ncbi.nlm.nih.gov/32917362/
6. Mujtaba, Ghulam, Bukhari, Ihtisham, Fatima, Amara, Naz, Sadaf. 2012. A p.C343S missense mutation in PJVK causes progressive hearing loss. In Gene, 504, 98-101. doi:10.1016/j.gene.2012.05.013. https://pubmed.ncbi.nlm.nih.gov/22617256/
7. Zheng, Zengzhang, Deng, Wanyan, Lou, Xiwen, Gong, Sitang, Liu, Xing. . Gasdermins: pore-forming activities and beyond. In Acta biochimica et biophysica Sinica, 52, 467-474. doi:10.1093/abbs/gmaa016. https://pubmed.ncbi.nlm.nih.gov/32294153/
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
Model Library
Model Library
Resources
Resources
Animal Quality
Animal Quality
Get Support
Get Support
Address:
2255 Martin Avenue, Suite E Santa Clara, CA 95050-2709, US
Tel:
800-921-8930 (8-6pm PST)
+1408-963-0306 (lnt’l)
Fax:
408-969-0338
Email:
animal-service@cyagen.com
service@cyagen.us
CRO Services
OncologyOphthalmologyNeuroscienceMetabolic & CardiovascularAutoimmune & InflammatoryGene TherapyAntibody Therapy
About Us
Corporate OverviewOur PartnersCareersContact Us
Social Media
Disclaimer: Pricing and availability of our products and services vary by region. Listed prices are applicable to the specific countries. Please contact us for more information.
Copyright © 2025 Cyagen. All rights reserved.
Privacy Policy
Site Map
Stay Updated with the Latest from Cyagen
Get the latest news on our research models, CRO services, scientific resources, and special offers—tailored to your research needs and delivered straight to your inbox.
Full Name
Email
Organization
Country
Areas of Interest