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C57BL/6JCya-Rnh1em1flox/Cya
Common Name:
Rnh1-flox
Product ID:
S-CKO-00595
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
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Basic Information
Strain Name
Rnh1-flox
Strain ID
CKOCMP-107702-Rnh1-B6J-VA
Gene Name
Rnh1
Product ID
S-CKO-00595
Gene Alias
RNH
Background
C57BL/6JCya
NCBI ID
107702
Modification
Conditional knockout
Chromosome
7
Phenotype
MGI:1195456
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Rnh1em1flox/Cya mice (Catalog S-CKO-00595) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000106033
NCBI RefSeq
NM_145135
Target Region
Exon 3~8
Size of Effective Region
~3.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Rnh1, also known as ribonuclease inhibitor 1 or angiogenin inhibitor 1, is a ubiquitously expressed leucine-rich repeat protein. It is an accessory part of the translation machinery. Rnh1 interacts with angiogenin (ANG) to inhibit its ribonucleolytic activity, and can also bind to 40S ribosomes to control translation. It is involved in multiple biological processes such as translation regulation, inflammasome activation, and iron homeostasis [1,2,7].

In mouse models, inactivation of rnh1 causes embryonically lethal anemia [5]. In humans, loss-of-function of RNH1 is associated with a multiorgan developmental disease including congenital cataracts, global developmental delay, myopathy, psychomotor deterioration, seizures, and periodic anemia. Patient fibroblasts with RNH1 deficiency are more sensitive to RNase A exposure [5]. Biallelic variants in RNH1 confer susceptibility to a subtype of acute, necrotizing encephalopathy in children [6]. In bladder cancer, low RNH1 expression is related to enhanced invasion and metastasis, and it can also predict immunotherapy response [3]. In lung adenocarcinoma, E2F1 binds to the RNH1 promoter to inhibit its expression, promoting tumor development [4].

In conclusion, Rnh1 plays essential roles in translation, cell development, and homeostasis. Studies on Rnh1 knockout mouse models have revealed its significance in various disease conditions such as anemia, multiorgan developmental diseases, and certain types of encephalopathy. Understanding Rnh1 provides insights into disease mechanisms and may offer potential therapeutic targets.

References:
1. Sarangdhar, Mayuresh Anant, Allam, Ramanjaneyulu. 2021. Angiogenin (ANG)-Ribonuclease Inhibitor (RNH1) System in Protein Synthesis and Disease. In International journal of molecular sciences, 22, . doi:10.3390/ijms22031287. https://pubmed.ncbi.nlm.nih.gov/33525475/
2. Bombaci, Giuseppe, Sarangdhar, Mayuresh Anant, Andina, Nicola, Maslowski, Kendle M, Allam, Ramanjaneyulu. 2022. LRR-protein RNH1 dampens the inflammasome activation and is associated with COVID-19 severity. In Life science alliance, 5, . doi:10.26508/lsa.202101226. https://pubmed.ncbi.nlm.nih.gov/35256513/
3. Chen, Sen, Ran, Jun, Fan, Zhouqian, Peng, Jian, Hu, Zuquan. 2023. Functional status analysis of RNH1 in bladder cancer for predicting immunotherapy response. In Scientific reports, 13, 12625. doi:10.1038/s41598-023-39827-7. https://pubmed.ncbi.nlm.nih.gov/37537337/
4. Zhao, Wenyue, Liu, Yang, Yang, Ying, Wang, Liming. 2024. New link between RNH1 and E2F1: regulates the development of lung adenocarcinoma. In BMC cancer, 24, 635. doi:10.1186/s12885-024-12392-6. https://pubmed.ncbi.nlm.nih.gov/38783241/
5. Hedberg-Oldfors, Carola, Mitra, Sanhita, Molinaro, Angela, Sterky, Fredrik H, Darin, Niklas. 2023. Ribonuclease inhibitor 1 (RNH1) deficiency cause congenital cataracts and global developmental delay with infection-induced psychomotor regression and anemia. In European journal of human genetics : EJHG, 31, 887-894. doi:10.1038/s41431-023-01327-7. https://pubmed.ncbi.nlm.nih.gov/36935417/
6. Shashi, Vandana, Schoch, Kelly, Ganetzky, Rebecca, Innes, A Micheil, Davis, Erica E. 2023. Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy. In Genetics in medicine : official journal of the American College of Medical Genetics, 25, 100897. doi:10.1016/j.gim.2023.100897. https://pubmed.ncbi.nlm.nih.gov/37191094/
7. Neu, Carolina, Beckers, Christian, Frank, Nadine, Kraemer, Sandra, Zechendorf, Elisabeth. 2024. Ribonuclease inhibitor 1 emerges as a potential biomarker and modulates inflammation and iron homeostasis in sepsis. In Scientific reports, 14, 14972. doi:10.1038/s41598-024-65778-8. https://pubmed.ncbi.nlm.nih.gov/38951571/
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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