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C57BL/6JCya-Ikbkgem1flox/Cya
Common Name:
Ikbkg-flox
Product ID:
S-CKO-03073
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
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Basic Information
Strain Name
Ikbkg-flox
Strain ID
CKOCMP-16151-Ikbkg-B6J-VA
Gene Name
Ikbkg
Product ID
S-CKO-03073
Gene Alias
1110037D23Rik; IKK[g]; NEMO
Background
C57BL/6JCya
NCBI ID
16151
Modification
Conditional knockout
Chromosome
X
Phenotype
MGI:1338074
Document
Click here to download >>
Application
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More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ikbkgem1flox/Cya mice (Catalog S-CKO-03073) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000004330
NCBI RefSeq
NM_001136067
Target Region
Exon 4
Size of Effective Region
~1.3 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Ikbkg, also known as IKBKG/NEMO (inhibitor of nuclear factor kappa B kinase regulatory subunit gamma), is essential for the NF-κB activation pathway [2,6]. The NF-κB pathway is involved in a variety of physiological and cellular processes, such as immunity, inflammation, cell proliferation, and survival [2].

Mutations in the Ikbkg gene are responsible for rare diseases like Anhidrotic Ectodermal Dysplasia with ImmunoDeficiency (EDA-ID) and Incontinentia Pigmenti (IP) [2]. In IP patients, Ikbkg mutations lead to a wide range of manifestations including skin, teeth, eyes, and central nervous system anomalies [3,4,5,7]. For instance, some IP patients have corpus callosum abnormalities which may be caused by Ikbkg mutations [3]. Additionally, in an atypical IP case, a novel germline missense mutation in Ikbkg led to persistent mucocutaneous hyperinflammation and immunodeficiency [8]. In colon cancer, RIPK2 promotes cancer progression by regulating BIRC3-mediated ubiquitination of Ikbkg, thus activating the NF-κB signaling pathway [9]. On the other hand, fluoride-induced down-regulation of Ikbkg gene expression inhibits hepatocytes senescence [1].

In conclusion, Ikbkg plays a crucial role in the NF-κB activation pathway, which is involved in multiple physiological processes. Its mutations are associated with rare genetic disorders like EDA-ID and IP, and it also has implications in conditions such as colon cancer and hepatocyte senescence. Research on Ikbkg using gene-based models helps to understand the molecular mechanisms underlying these diseases, potentially paving the way for new therapeutic strategies.

References:
1. Wu, C X, Mao, C Y, Deng, J, Hu, X X, Qi, X L. 2021. Fluoride induced down-regulation of IKBKG Gene expression inhibits hepatocytes senescence. In Journal of trace elements in medicine and biology : organ of the Society for Minerals and Trace Elements (GMS), 69, 126896. doi:10.1016/j.jtemb.2021.126896. https://pubmed.ncbi.nlm.nih.gov/34763226/
2. Fusco, Francesca, Pescatore, Alessandra, Conte, Matilde Immacolata, Lioi, Maria Brigida, Ursini, Matilde Valeria. 2015. EDA-ID and IP, two faces of the same coin: how the same IKBKG/NEMO mutation affecting the NF-κB pathway can cause immunodeficiency and/or inflammation. In International reviews of immunology, 34, 445-59. doi:10.3109/08830185.2015.1055331. https://pubmed.ncbi.nlm.nih.gov/26269396/
3. Minić, Snežana, Cerovac, Nataša, Novaković, Ivana, Popadić, Svetlana, Trpinac, Dušan. 2023. The Impact of the IKBKG Gene on the Appearance of the Corpus Callosum Abnormalities in Incontinentia Pigmenti. In Diagnostics (Basel, Switzerland), 13, . doi:10.3390/diagnostics13071300. https://pubmed.ncbi.nlm.nih.gov/37046518/
4. Chen, Huaqing, Ji, Xiaojuan, Lai, Yun, Wan, Chunlei, Li, Longnian. 2023. Novel IKBKG gene mutations in incontinentia pigmenti: report of two cases. In Frontiers in medicine, 10, 1303590. doi:10.3389/fmed.2023.1303590. https://pubmed.ncbi.nlm.nih.gov/38173938/
5. Cammarata-Scalisi, F, Fusco, F, Ursini, M V. 2019. Incontinentia Pigmenti. In Actas dermo-sifiliograficas, 110, 273-278. doi:10.1016/j.ad.2018.10.004. https://pubmed.ncbi.nlm.nih.gov/30660327/
6. Qiu, Yueping, Wang, Jincheng, Li, Hui, He, Qiaojun, Weng, Qinjie. 2021. Emerging views of OPTN (optineurin) function in the autophagic process associated with disease. In Autophagy, 18, 73-85. doi:10.1080/15548627.2021.1908722. https://pubmed.ncbi.nlm.nih.gov/33783320/
7. Rosser, Tena. 2024. Incontinentia pigmenti. In Seminars in pediatric neurology, 51, 101156. doi:10.1016/j.spen.2024.101156. https://pubmed.ncbi.nlm.nih.gov/39389657/
8. Mou, Wenjun, Zhao, Zhipeng, Gao, Liwei, He, Jianxin, Gui, Jingang. 2023. An Atypical Incontinentia Pigmenti Female with Persistent Mucocutaneous Hyperinflammation and Immunodeficiency Caused by a Novel Germline IKBKG Missense Mutation. In Journal of clinical immunology, 43, 2165-2180. doi:10.1007/s10875-023-01564-x. https://pubmed.ncbi.nlm.nih.gov/37831401/
9. Zhang, Zhiyong, Pan, Yan, Zhao, Yan, Lu, Guifang, He, Shuixiang. 2023. RIPK2 promotes the progression of colon cancer by regulating BIRC3-mediated ubiquitination of IKBKG. In Experimental cell research, 429, 113644. doi:10.1016/j.yexcr.2023.113644. https://pubmed.ncbi.nlm.nih.gov/37211186/
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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