C57BL/6JCya-Ppp1r13lem1flox/Cya
Common Name:
Ppp1r13l-flox
Product ID:
S-CKO-10761
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Ppp1r13l-flox
Strain ID
CKOCMP-333654-Ppp1r13l-B6J-VA
Gene Name
Product ID
S-CKO-10761
Gene Alias
IASPP; Nkip1; wa3
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
7
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ppp1r13lem1flox/Cya mice (Catalog S-CKO-10761) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000047621
NCBI RefSeq
NM_001010836
Target Region
Exon 4~7
Size of Effective Region
~2.8 kb
Detailed Document
Overview of Gene Research
Ppp1r13l, also known as inhibitor of apoptosis-stimulating protein of p53 (iASPP), is a conserved inhibitor of p53, selectively regulating a subset of p53 target genes. It is involved in multiple biological processes, including apoptosis, regulation of desmosomes, and inflammatory pathways [1,2].
In cervical cancer, PPP1R13L promotes cell proliferation, epithelial-mesenchymal transition, cycle progression, and glycolysis via the PTEN/AKT/mTOR pathway. It regulates the transcription of PTEN through its Ank-SH3 domain interaction with p53 family members, p53 and p63 [1].
In the context of paediatric dilated cardiomyopathy, biallelic variants in PPP1R13L have been identified in multiple children. The protein encoded by PPP1R13L, iASPP, acts as a regulator of desmosomes and is implicated in inflammatory pathways, and its deficiency leads to progressive DCM, often resulting in death or transplant [2].
In lung adenocarcinoma, PPP1R13L, as an oncoprotein, along with transcription factor SP1, forms a feedback loop to regulate nicotine-induced epithelial-mesenchymal transition [3]. High iASPP (PPP1R13L) expression in acute myeloid leukemia (AML) is an independent predictor of adverse clinical outcome, and its attenuation reduces leukemic blast proliferation and increases apoptosis susceptibility [4].
In summary, Ppp1r13l plays crucial roles in various biological processes and disease conditions. In cancer, it can promote cancer progression, such as in cervical and lung cancers, and is related to adverse outcomes in AML. In cardiomyopathies, biallelic variants in Ppp1r13l are associated with severe paediatric dilated cardiomyopathy and arrhythmogenic cardiomyopathy. The study of Ppp1r13l using genetic models helps to understand its functions in these disease areas and may provide potential therapeutic targets.
References:
1. Wang, Anjin, Liu, Xuelian, Liang, Ziyan, Wang, Hua, Cai, Hongbin. 2025. PPP1R13L drives cervical cancer progression by suppressing p63-mediated PTEN transcription. In Cellular and molecular life sciences : CMLS, 82, 97. doi:10.1007/s00018-025-05598-9. https://pubmed.ncbi.nlm.nih.gov/40014097/
2. Robinson, H K, Zaklyazminskaya, E, Povolotskaya, I, Turner, C L, Ellard, S. 2020. Biallelic variants in PPP1R13L cause paediatric dilated cardiomyopathy. In Clinical genetics, 98, 331-340. doi:10.1111/cge.13812. https://pubmed.ncbi.nlm.nih.gov/32666529/
3. Zhang, Hongchao, Zhang, Guopei, Zhang, Jingyue, Yang, Jinghua, Lu, Xiaobo. 2022. Transcription factor SP1 and oncoprotein PPP1R13L regulate nicotine-induced epithelial-mesenchymal transition in lung adenocarcinoma via a feedback loop. In Biochemical pharmacology, 206, 115344. doi:10.1016/j.bcp.2022.115344. https://pubmed.ncbi.nlm.nih.gov/36372331/
4. Bajrami Saipi, Mihada, Ruiba, Alessia, Schittenhelm, Marcus Matthias, Christoph, Driessen, Kampa-Schittenhelm, Kerstin Maria. 2024. High iASPP (PPP1R13L) expression is an independent predictor of adverse clinical outcome in acute myeloid leukemia (AML). In Cell death & disease, 15, 869. doi:10.1038/s41419-024-07190-8. https://pubmed.ncbi.nlm.nih.gov/39616159/
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