C57BL/6JCya-Pkd1em1/Cya
Common Name:
Pkd1-KO
Product ID:
S-KO-17342
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Pkd1-KO
Strain ID
KOCMP-18763-Pkd1-B6J-VA
Gene Name
Product ID
S-KO-17342
Gene Alias
PC1; mFLJ00285
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
17
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pkd1em1/Cya mice (Catalog S-KO-17342) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000035565
NCBI RefSeq
NM_013630
Target Region
Exon 2~16
Size of Effective Region
~13.5 kb
Detailed Document
Overview of Gene Research
Pkd1, encoding Polycystin-1, is one of the key genes related to autosomal dominant polycystic kidney disease (ADPKD), one of the most common monogenetic disorders. Mutations in Pkd1 and Pkd2 account for most ADPKD cases. Pkd1 contains a voltage-gated ion channel (VGIC) fold, which interacts with Pkd2 to form a non-canonical transient receptor potential (TRP) channel architecture [1].
PKD1 loss-of-function studies have shown diverse impacts. In murine models, Pkd1 re-expression from hybrid therapeutic constructs can ameliorate or eliminate polycystic kidney disease (PKD), indicating that Pkd1 intragenic sequences regulate expression levels and spatiotemporal patterns [3]. Pkd1 haploinsufficiency accelerated liver regeneration after partial hepatectomy, and Pkd1 loss protected against fatty liver disease, steatosis, and glucose intolerance, selectively increasing mTOR signaling without SREBP-1c activation [2]. In a porcine ADPKD model, PKD1 deficiency induced bronchiectasis by suppressing E-cadherin expression in the airway epithelial barrier, altering extracellular matrix components, and causing airway remodeling [4]. Also, in mice, Pkd1 interacts with Wnt5a in the non-canonical WNT signaling pathway to control lymphatic vascular morphogenesis, with loss of either gene suppressing the phenotypes seen in the other's knockout [5].
In conclusion, Pkd1 is crucial in multiple biological processes. Through gene-targeted strategies in animal models, its role in ADPKD, liver diseases, bronchiectasis, and lymphatic vascular development has been revealed. These findings provide insights into disease mechanisms and potential therapeutic targets related to Pkd1-associated pathologies.
References:
1. Su, Qiang, Hu, Feizhuo, Ge, Xiaofei, Mei, Changlin, Shi, Yigong. 2018. Structure of the human PKD1-PKD2 complex. In Science (New York, N.Y.), 361, . doi:10.1126/science.aat9819. https://pubmed.ncbi.nlm.nih.gov/30093605/
2. Zhu, Min, Wang, Yunguan, Lu, Tianshi, Wang, Tao, Zhu, Hao. 2024. PKD1 mutant clones within cirrhotic livers inhibit steatohepatitis without promoting cancer. In Cell metabolism, 36, 1711-1725.e8. doi:10.1016/j.cmet.2024.05.015. https://pubmed.ncbi.nlm.nih.gov/38901424/
3. Kurbegovic, Almira, Pacis, Rey Christian, Trudel, Marie. 2023. Modeling Pkd1 gene-targeted strategies for correction of polycystic kidney disease. In Molecular therapy. Methods & clinical development, 29, 366-380. doi:10.1016/j.omtm.2023.03.016. https://pubmed.ncbi.nlm.nih.gov/37214311/
4. Wang, Runming, Li, Wenya, Dai, Haiting, Hu, Xiaoxiang, Xing, Yiming. 2022. PKD1 deficiency induces Bronchiectasis in a porcine ADPKD model. In Respiratory research, 23, 292. doi:10.1186/s12931-022-02214-3. https://pubmed.ncbi.nlm.nih.gov/36309681/
5. Chau, Tevin C Y, Baek, Sungmin, Coxam, Baptiste, François, Mathias, Hogan, Benjamin M. 2021. Pkd1 and Wnt5a genetically interact to control lymphatic vascular morphogenesis in mice. In Developmental dynamics : an official publication of the American Association of Anatomists, 251, 336-349. doi:10.1002/dvdy.390. https://pubmed.ncbi.nlm.nih.gov/34174014/
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