C57BL/6JCya-Nfkb1em1flox/Cya
Common Name
Nfkb1-flox
Product ID
S-CKO-03945
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-18033-Nfkb1-B6J-VA
Status
When using this mouse strain in a publication, please cite “Nfkb1-flox Mouse (Catalog S-CKO-03945) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Nfkb1-flox
Strain ID
CKOCMP-18033-Nfkb1-B6J-VA
Gene Name
Product ID
S-CKO-03945
Gene Alias
p50, p105, NF-KB1, p50/p105, NF-kappaB, NF-kappaB1
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 3 (Mouse)
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Transcript ID
ENSMUST00000029812
NCBI Transcript ID
NM_008689
Target Region
Exon 4
Size of Effective Region
~1.1 kb
Overview of Gene Research
NFKB1, encoding the p105/p50 subunits of NF-κB, is a key transcription factor. The NF-κB signaling pathway, in which NFKB1 is involved, governs crucial cellular processes such as immune responses, apoptosis, and cellular homeostasis [2,5]. Aberrant activation of this pathway is associated with carcinogenesis [2].
NFKB1-related disease, often caused by heterozygous NFKB1 mutations, presents as an inborn error of immunity with immune dysregulation rather than a simple primary immunodeficiency. Clinical features include hypogammaglobulinemia, reduced switched memory B cells, respiratory and gastrointestinal infections, as well as high frequencies of autoimmunity, lymphoproliferation, non-infectious enteropathy, opportunistic infections, autoinflammation, and malignancy [1]. Truncating monoallelic loss-of-function variants of NFKB1 can lead to combined NLRP3 inflammasome activation and type I interferon signaling, predisposing patients to necrotizing fasciitis [3]. In addition, the NFKB1-94ins/delATTG polymorphism is associated with a decreased risk of overall cancer, and this association holds for some specific cancers like lung, nasopharyngeal, prostate, ovarian, and oral squamous cell carcinoma [4].
In conclusion, NFKB1 is vital for normal immune function and maintaining cellular homeostasis. Studies on NFKB1-related mutations in patients and animal models have revealed its complex roles in immunodeficiency, autoimmunity, autoinflammation, and cancer. Understanding NFKB1 is crucial for developing therapeutic strategies for these related diseases.
References：
1. Lorenzini, Tiziana, Fliegauf, Manfred, Klammer, Nils, Warnatz, Klaus, Grimbacher, Bodo. 2020. Characterization of the clinical and immunologic phenotype and management of 157 individuals with 56 distinct heterozygous NFKB1 mutations. In The Journal of allergy and clinical immunology, 146, 901-911. doi:10.1016/j.jaci.2019.11.051. https://pubmed.ncbi.nlm.nih.gov/32278790/
2. Concetti, Julia, Wilson, Caroline L. 2018. NFKB1 and Cancer: Friend or Foe? In Cells, 7, . doi:10.3390/cells7090133. https://pubmed.ncbi.nlm.nih.gov/30205516/
3. Nurmi, Katariina, Silventoinen, Kristiina, Keskitalo, Salla, Seppänen, Mikko R J, Eklund, Kari K. 2024. Truncating NFKB1 variants cause combined NLRP3 inflammasome activation and type I interferon signaling and predispose to necrotizing fasciitis. In Cell reports. Medicine, 5, 101503. doi:10.1016/j.xcrm.2024.101503. https://pubmed.ncbi.nlm.nih.gov/38593810/
4. Fu, Wen, Zhuo, Zhen-Jian, Chen, Yung-Chang, He, Jing, Liu, Guo-Chang. . NFKB1 -94insertion/deletion ATTG polymorphism and cancer risk: Evidence from 50 case-control studies. In Oncotarget, 8, 9806-9822. doi:10.18632/oncotarget.14190. https://pubmed.ncbi.nlm.nih.gov/28039461/
5. Cartwright, Tyrell, Perkins, Neil D, L Wilson, Caroline. 2016. NFKB1: a suppressor of inflammation, ageing and cancer. In The FEBS journal, 283, 1812-22. doi:10.1111/febs.13627. https://pubmed.ncbi.nlm.nih.gov/26663363/
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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