C57BL/6JCya-Atp13a1em1flox/Cya
Common Name
Atp13a1-flox
Product ID
S-CKO-03508
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-170759-Atp13a1-B6J-VA
When using this mouse strain in a publication, please cite “Atp13a1-flox Mouse (Catalog S-CKO-03508) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Atp13a1-flox
Strain ID
CKOCMP-170759-Atp13a1-B6J-VA
Gene Name
Product ID
S-CKO-03508
Gene Alias
catp, Atp13a, Cgi152
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 8
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000034326
NCBI RefSeq
NM_133224
Target Region
Exon 2~3
Size of Effective Region
~0.9 kb
Overview of Gene Research
Atp13a1, a member of the P5A-ATPase family, is located in the endoplasmic reticulum (ER). It plays crucial roles in preventing the accumulation of mislocalized and misoriented proteins, facilitating the topogenesis of certain proteins, and is involved in pathways such as ER-associated degradation (ERAD) and antiviral innate immunity [1,2,3]. It is also potentially related to intellectual disability and modulates major histocompatibility complex I-related protein 1 (MR1)-mediated antigen presentation [4,5]. Mouse models are valuable for studying its functions.
In gene knockout studies, Atp13a1 deficiency abolishes RIG-I-mediated antiviral innate immune response due to compromised MAVS stability and protease-mediated degradation of MAVS. Homozygous Atp13a1 knockout mice result in developmental retardation and embryonic lethality, while myeloid-specific Atp13a1-deficient (conditional knockout) mice are viable but susceptible to RNA virus infection [3].
In conclusion, Atp13a1 is essential for maintaining protein homeostasis in the ER, ensuring proper protein folding and topogenesis. Its role in antiviral innate immunity is also significant. The use of Atp13a1 KO and CKO mouse models has provided insights into its function in the context of antiviral responses and highlighted its potential importance in understanding certain disease conditions related to immune function and development.
References：
1. McKenna, Michael J, Adams, Benjamin M, Chu, Vincent, Paulo, Joao A, Shao, Sichen. 2022. ATP13A1 prevents ERAD of folding-competent mislocalized and misoriented proteins. In Molecular cell, 82, 4277-4289.e10. doi:10.1016/j.molcel.2022.09.035. https://pubmed.ncbi.nlm.nih.gov/36283413/
2. Ji, Jia, Cui, Meng-Ke, Zou, Rong, Li, Jiqiang, Zhang, Zai-Rong. 2024. An ATP13A1-assisted topogenesis pathway for folding multi-spanning membrane proteins. In Molecular cell, 84, 1917-1931.e15. doi:10.1016/j.molcel.2024.04.010. https://pubmed.ncbi.nlm.nih.gov/38723633/
3. Zhang, Rui, Hou, Xianteng, Wang, Changwan, Jiang, Yingbo, Hou, Fajian. 2022. The Endoplasmic Reticulum ATP13A1 is Essential for MAVS-Mediated Antiviral Innate Immunity. In Advanced science (Weinheim, Baden-Wurttemberg, Germany), 9, e2203831. doi:10.1002/advs.202203831. https://pubmed.ncbi.nlm.nih.gov/36216581/
4. Anazi, Shams, Maddirevula, Sateesh, Salpietro, Vincenzo, Faqeih, Eissa, Alkuraya, Fowzan S. 2017. Expanding the genetic heterogeneity of intellectual disability. In Human genetics, 136, 1419-1429. doi:10.1007/s00439-017-1843-2. https://pubmed.ncbi.nlm.nih.gov/28940097/
5. Kulicke, Corinna A, De Zan, Erica, Hein, Zeynep, Cerundolo, Vincenzo, Salio, Mariolina. 2021. The P5-type ATPase ATP13A1 modulates major histocompatibility complex I-related protein 1 (MR1)-mediated antigen presentation. In The Journal of biological chemistry, 298, 101542. doi:10.1016/j.jbc.2021.101542. https://pubmed.ncbi.nlm.nih.gov/34968463/
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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