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C57BL/6JCya-Pkp2em1/Cya
Common Name:
Pkp2-KO
Product ID:
S-KO-15719
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Pkp2-KO
Strain ID
KOCMP-67451-Pkp2-B6J-VA
Gene Name
Pkp2
Product ID
S-KO-15719
Gene Alias
1200008D14Rik; 1200012P04Rik; Pkp2l
Background
C57BL/6JCya
NCBI ID
67451
Modification
Conventional knockout
Chromosome
16
Phenotype
MGI:1914701
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pkp2em1/Cya mice (Catalog S-KO-15719) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000039408
NCBI RefSeq
NM_026163.2
Target Region
Exon 2~3
Size of Effective Region
~991 bp
Detailed Document
Click here to download >>
Overview of Gene Research
PKP2, or plakophilin-2, is a component of the desmosome complex, playing a crucial role in cell-cell adhesion [2]. The desmosome is important for maintaining the integrity of tissues, especially in the heart where proper cell-cell adhesion is vital for normal cardiac function.

In arrhythmogenic cardiomyopathy (ACM), which is characterized by lethal arrhythmias and sudden cardiac death, PKP2 is the most frequently affected gene. AAV-mediated delivery of PKP2 in PKP2 mutant induced pluripotent stem cell-derived cardiomyocytes restored cardiac PKP2 levels and those of other junctional proteins, improved sodium conduction, enhanced contractile function, and normalized contraction kinetics. In vivo, treating heterozygous Pkp2c.1755delA knock-in mice with AAV9-PKP2 corroborated the recovery of desmosomal integrity and cardiac function, and long-term treatment prevented cardiac dysfunction in 12-month-old Pkp2c.1755delA/WT mice [1]. In addition, adult hearts deficient in PKP2 show loss of nuclear envelope integrity, increased oxidant production, and DNA damage, key substrates in the molecular pathology of ACM [3].

In summary, PKP2 is essential for cell-cell adhesion, especially in the heart. Gene-knockout and related mouse models have revealed its critical role in arrhythmogenic cardiomyopathy, showing that restoring PKP2 can potentially rescue the arrhythmic substrate and cardiac function. These findings encourage the exploration of PKP2 gene therapy for ACM patients with PKP2 haploinsufficiency [1].

References:

1. Kyriakopoulou, Eirini, Versteeg, Danielle, de Ruiter, Hesther, Giacca, Mauro, van Rooij, Eva. 2023. Therapeutic efficacy of AAV-mediated restoration of PKP2 in arrhythmogenic cardiomyopathy. In Nature cardiovascular research, 2, 1262-1276. doi:10.1038/s44161-023-00378-9. https://pubmed.ncbi.nlm.nih.gov/38665939/

2. Novelli, Valeria, Malkani, Kabir, Cerrone, Marina. 2018. Pleiotropic Phenotypes Associated With PKP2 Variants. In Frontiers in cardiovascular medicine, 5, 184. doi:10.3389/fcvm.2018.00184. https://pubmed.ncbi.nlm.nih.gov/30619891/

3. Pérez-Hernández, Marta, van Opbergen, Chantal J M, Bagwan, Navratan, Delmar, Mario, Lundby, Alicia. 2022. Loss of Nuclear Envelope Integrity and Increased Oxidant Production Cause DNA Damage in Adult Hearts Deficient in PKP2: A Molecular Substrate of ARVC. In Circulation, 146, 851-867. doi:10.1161/CIRCULATIONAHA.122.060454. https://pubmed.ncbi.nlm.nih.gov/35959657/

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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