C57BL/6NCya-Il6stem1flox/Cya
Common Name:
Il6st-flox
Product ID:
S-CKO-03111
Background:
C57BL/6NCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Il6st-flox
Strain ID
CKOCMP-16195-Il6st-B6N-VA
Gene Name
Product ID
S-CKO-03111
Gene Alias
5133400A03Rik; CD130; D13Ertd699e; gp130
Background
C57BL/6NCya
NCBI ID
Modification
Conditional knockout
Chromosome
13
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6NCya-Il6stem1flox/Cya mice (Catalog S-CKO-03111) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000183663
NCBI RefSeq
NM_010560
Target Region
Exon 4~5
Size of Effective Region
~2.1 kb
Detailed Document
Overview of Gene Research
Il6st, also known as interleukin 6 cytokine family signal transducer or gp130, is a key component of the receptor complex for the IL-6 family of cytokines, including IL-6, IL-11, LIF, etc. These cytokines activate various intracellular signalling pathways such as JAK/STAT, MAPK and PI3K, which are essential for immune homeostasis, haematopoiesis, inflammation, development and metabolism [2,3].
Mutations in Il6st can lead to various diseases. Dominant-negative mutations in human Il6st underlie autosomal dominant hyper-IgE syndrome (AD-HIES), with patients suffering from cold staphylococcal lesions, mucocutaneous candidiasis, severe allergy, and skeletal abnormalities [5,6]. A mosaic Il6st variant inducing constitutive GP130 cytokine receptor signalling causes neonatal onset immunodeficiency with autoinflammation and dysmorphy [7]. In hepatocellular carcinoma, impaired degradation of IL6ST by chaperone-mediated autophagy promotes cell proliferation and migration [1]. In Parkinson's disease, IL6ST is involved in the α-Synuclein-induced neuroinflammation injury through the IL6ST-AS/STAT3/HIF-1α axis [4].
In conclusion, Il6st is crucial for the normal function of the IL-6 cytokine family signalling pathways. Studies on Il6st-related mouse models (implied by the human disease-causing mutations) could potentially help understand its role in diseases like AD-HIES, neonatal immunodeficiency, hepatocellular carcinoma, and Parkinson's disease, providing insights for diagnosis and treatment strategies.
References：
1. Desideri, Enrico, Castelli, Serena, Dorard, Coralie, Ciriolo, Maria Rosa, Baccarini, Manuela. 2022. Impaired degradation of YAP1 and IL6ST by chaperone-mediated autophagy promotes proliferation and migration of normal and hepatocellular carcinoma cells. In Autophagy, 19, 152-162. doi:10.1080/15548627.2022.2063004. https://pubmed.ncbi.nlm.nih.gov/35435804/
2. Nicola, Nicos A, Babon, Jeffrey J. 2015. Leukemia inhibitory factor (LIF). In Cytokine & growth factor reviews, 26, 533-44. doi:10.1016/j.cytogfr.2015.07.001. https://pubmed.ncbi.nlm.nih.gov/26187859/
3. Jones, Simon A, Jenkins, Brendan J. . Recent insights into targeting the IL-6 cytokine family in inflammatory diseases and cancer. In Nature reviews. Immunology, 18, 773-789. doi:10.1038/s41577-018-0066-7. https://pubmed.ncbi.nlm.nih.gov/30254251/
4. Lin, Danyu, Zhang, Han, Zhang, Jieli, Jing, Xiuna, Tao, Enxiang. 2023. α-Synuclein Induces Neuroinflammation Injury through the IL6ST-AS/STAT3/HIF-1α Axis. In International journal of molecular sciences, 24, . doi:10.3390/ijms24021436. https://pubmed.ncbi.nlm.nih.gov/36674945/
5. Béziat, Vivien, Tavernier, Simon J, Chen, Yin-Huai, Casanova, Jean-Laurent, Puel, Anne. . Dominant-negative mutations in human IL6ST underlie hyper-IgE syndrome. In The Journal of experimental medicine, 217, . doi:10.1084/jem.20191804. https://pubmed.ncbi.nlm.nih.gov/32207811/
6. Arlabosse, Tiphaine, Materna, Marie, Riccio, Orbicia, Béziat, Vivien, Candotti, Fabio. 2023. New Dominant-Negative IL6ST Variants Expand the Immunological and Clinical Spectrum of GP130-Dependent Hyper-IgE Syndrome. In Journal of clinical immunology, 43, 1566-1580. doi:10.1007/s10875-023-01517-4. https://pubmed.ncbi.nlm.nih.gov/37273120/
7. Materna-Kiryluk, Anna, Pollak, Agnieszka, Gawalski, Karol, Nowis, Dominika, Ploski, Rafal. . Mosaic IL6ST variant inducing constitutive GP130 cytokine receptor signaling as a cause of neonatal onset immunodeficiency with autoinflammation and dysmorphy. In Human molecular genetics, 30, 226-233. doi:10.1093/hmg/ddab035. https://pubmed.ncbi.nlm.nih.gov/33517393/
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