mProX™ Human IL11RA Stable Cell Line

Product Information

Target Family

Kinases/Enzyme

Target Protein Species

Human

Host Cell Type

HEK293;CHO-K1;LO-2

Target Classification

Kinase Cell Lines

Target Research Area

Orphan Receptor Research

Related Diseases

Craniosynostosis And Dental Anomalies; Craniosynostosis

Gene ID

Human:3590

UniProt ID

Human:Q14626

Product Properties

Biosafety Level

Level 1

Activity

Yes

Quantity

10⁶ cells per vial

Applications

IL11RA, also known as interleukin-11 receptor alpha, is involved in various medical conditions. In craniosynostosis patients, IL11RA is one of the genes with a high diagnostic yield when testing for pathogenic variants. It has been identified as a causative variant in craniosynostosis cases, leading to premature fusion of the skull sutures. IL11RA has also been implicated in ovarian carcinomatosis, where it is expressed in carcinoma-associated mesothelial cells (CAMs) and can be targeted with antibody-drug conjugates for treatment. In hepatic fibrosis, IL-11 derived from hepatic stellate cells promotes hepatocyte injury, suggesting that targeting IL11RA secretion may be a potential therapeutic strategy. Additionally, IL11RA gene expression has been found to be higher in primary melanoma tissues with liver metastasis, indicating its potential role in organ-specific metastasis. Overall, IL11RA has implications in craniosynostosis, ovarian carcinomatosis, hepatic fibrosis, and melanoma liver metastasis.

Protocols

Please visit our protocols page.

Customer Reviews

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FAQ

chat Jordan Miller (Verified Customer)

How is IL11RA related to craniosynostosis syndromes? Apr 22 2022

chat Patrick Liam (Creative Biolabs Scientific Support)

IL11RA mutations are associated with syndromic forms of craniosynostosis, particularly pansynostosis. Apr 22 2022

chat Alex Smith (Verified Customer)

What is the significance of IL11RA in cancer research? Jul 19 2020

chat Patrick Liam (Creative Biolabs Scientific Support)

Differential expression of IL11RA has been observed in cancers like vulvar carcinoma, suggesting its potential role in cancer development. Jul 19 2020

Published Data

Fig.1 In LO-2 cells exposed to TGF-β1-treated LX-2 supernatant, the concurrent inhibition of IL-11 and IL11RA demonstrates a substantial decrease in cellular apoptosis, underscoring the notable impact of dual gene silencing on this crucial biological process.

Apoptosis rates in LO-2 cells underwent distinct changes when exposed to various treatments, including CM Original TGF-β1 (+: stimulation of LO-2 cells with the supernatant of LX-2 and IL-11 induced by 10 ng/mL TGF-β1), CM Original TGF-β1 alone, siRNA/IL11RA (+: IL-11RA knockdown in LO-2 cells), and anti-IL-11 (+: cell supernatant treated with IL-11 neutralizing antibody), as assessed through flow cytometry analysis. '-' signifies the absence of treatment.

Ref: Jiang, Ling-Feng, et al. "The effect of hepatic stellate cell derived-IL-11 on hepatocyte injury in hepatic fibrosis." Life Sciences 330 (2023): 121974.

Pubmed: 37495078

DOI: 10.1016/j.lfs.2023.121974

Research Highlights

Gaillard, Linda. et al. "Genetic diagnostic yield in an 11-year cohort of craniosynostosis patients." European journal of medical genetics, 2023.
The study focuses on the diagnostic yield of genetic testing in children with craniosynostosis, a condition in which the skull bones fuse together prematurely. It can occur on its own or with other medical conditions. The study analyzed data from 1020 patients over 11 years and found that 35% had genetic variants that could cause craniosynostosis. Syndromic cases had a higher diagnostic rate (62%) compared to non-syndromic cases (6%). Traditional genetic testing methods such as Sanger sequencing and microarray showed a yield of 11% and 8%, respectively. The implementation of whole exome sequencing (WES) in 2015 showed a yield of 10%. In cases where the cause was still unknown, more extensive genetic testing was recommended. The authors suggest considering other forms of genetic testing if WES and microarray testing do not provide a diagnosis. However, for parents who are uncomfortable with extensive testing, targeted panels could also be considered. In conclusion, genetic testing is crucial for identifying potential genetic causes in craniosynostosis patients, and further advancements in testing methods may improve diagnostic yield.
Gaillard, Linda. et al. "Genetic diagnostic yield in an 11-year cohort of craniosynostosis patients." European journal of medical genetics, 2023.
Pubmed: 37716645 DOI: 10.1016/j.ejmg.2023.104843

Ahmad, Ilyas. et al. "The interleukin-11 receptor variant p.W307R results in craniosynostosis in humans." Scientific reports, 2023.
Craniosynostosis is a condition marked by the premature fusion and hardening of one or more sutures in the skull, often leading to facial and cranial abnormalities. When brain growth exceeds available space in the skull, it can result in developmental delays or cognitive issues. This study focuses on two siblings with craniosynostosis, both carrying a homozygous missense pathogenic variant in the IL11RA gene (c.919 T> C; p.W307R). These patients exhibit craniosynostosis, exophthalmos, delayed tooth eruption, mild platybasia, and basilar invagination. The p.W307R variant affects the IL-11R's structural integrity, causing impaired maturation, no cell surface transport, and intracellular retention, ultimately leading to a loss-of-function mutation. In conclusion, this previously unreported IL-11R-W307R variant is likely the root cause of craniosynostosis in these patients.
Ahmad, Ilyas. et al. "The interleukin-11 receptor variant p.W307R results in craniosynostosis in humans." Scientific reports, 2023.
Pubmed: 37596289 DOI: 10.1038/s41598-023-39466-y