C57BL/6JCya-Dbr1em1flox/Cya
Common Name:
Dbr1-flox
Product ID:
S-CKO-18167
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Dbr1-flox
Strain ID
CKOCMP-83703-Dbr1-B6J-VB
Gene Name
Product ID
S-CKO-18167
Gene Alias
-
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
9
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Dbr1em1flox/Cya mice (Catalog S-CKO-18167) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000066650
NCBI RefSeq
NM_031403
Target Region
Exon 3~5
Size of Effective Region
~2.4 kb
Detailed Document
Overview of Gene Research
Dbr1, short for RNA DEBRANCHING ENZYME 1, is a rate-limiting enzyme in the lariat intronic RNA (lariRNA) decay process. During pre-mRNA splicing, introns are removed as branched lariat RNAs, and Dbr1 debranches them in the rate-limiting step of lariat turnover. This process is crucial for intron recycling and likely regulates the production of translation-competent mRNAs [2,3,5].
In plant studies, sequestration of Dbr1 into stress granules (SGs) by SICKLE (SIC) enhances lariRNA accumulation, which promotes the transcription of heat-stress tolerance genes, highlighting its role in plant heat-stress responses [1]. In human cells, Dbr1 depletion leads to a 20-fold increase in lariats and increased exon skipping, indicating its role in proper splicing and spliceosome recycling [3,5]. Inherited DBR1 deficiency in humans is associated with brainstem viral encephalitis, as DBR1-deficient cells have impaired stress granule assembly, which in turn impairs PKR-mediated antiviral immunity [4,6]. A founder DBR1 variant also causes a lethal form of congenital ichthyosis [7]. In esophageal squamous cell carcinoma, lower DBR1 expression is associated with a worse prognosis [8].
In summary, Dbr1 is essential for lariat intronic RNA decay, which impacts mRNA processing, intron turnover, and splicing. Studies on Dbr1-related genetic models, such as human DBR1-deficient cases, have revealed its importance in various biological processes and disease conditions, including plant heat-stress tolerance, viral encephalitis, congenital ichthyosis, and esophageal squamous cell carcinoma prognosis.
References:
1. Wu, Chengyun, Wang, Xingsong, Li, Yan, Song, Chun-Peng, Hu, Zhubing. 2024. Sequestration of DBR1 to stress granules promotes lariat intronic RNAs accumulation for heat-stress tolerance. In Nature communications, 15, 7696. doi:10.1038/s41467-024-52034-w. https://pubmed.ncbi.nlm.nih.gov/39227617/
2. Mohanta, Arundhati, Chakrabarti, Kausik. 2020. Dbr1 functions in mRNA processing, intron turnover and human diseases. In Biochimie, 180, 134-142. doi:10.1016/j.biochi.2020.10.003. https://pubmed.ncbi.nlm.nih.gov/33038423/
3. Buerer, Luke, Clark, Nathaniel E, Welch, Anastasia, Mosammaparast, Nima, Fairbrother, William G. 2024. The debranching enzyme Dbr1 regulates lariat turnover and intron splicing. In Nature communications, 15, 4617. doi:10.1038/s41467-024-48696-1. https://pubmed.ncbi.nlm.nih.gov/38816363/
4. Chan, Yi-Hao, Lundberg, Vanja, Le Pen, Jérémie, Ekwall, Olov, Zhang, Shen-Ying. 2024. SARS-CoV-2 brainstem encephalitis in human inherited DBR1 deficiency. In The Journal of experimental medicine, 221, . doi:10.1084/jem.20231725. https://pubmed.ncbi.nlm.nih.gov/39023559/
5. Buerer, Luke, Clark, Nathaniel E, Welch, Anastasia, Mosammaparast, Nima, Fairbrother, William G. 2023. The debranching enzyme Dbr1 regulates lariat turnover and intron splicing. In Research square, , . doi:10.21203/rs.3.rs-2931976/v1. https://pubmed.ncbi.nlm.nih.gov/37398028/
6. Ru, Shuo, Tang, Sisi, Xu, Hui, Zhang, Shen-Ying, Gao, Daxing. 2024. Human DBR1 deficiency impairs stress granule-dependent PKR antiviral immunity. In The Journal of experimental medicine, 222, . doi:10.1084/jem.20240010. https://pubmed.ncbi.nlm.nih.gov/39636299/
7. Shamseldin, Hanan E, Sadagopan, Mukunth, Martini, Javier, Bertoli-Avella, Aida, Alkuraya, Fowzan S. 2023. A founder DBR1 variant causes a lethal form of congenital ichthyosis. In Human genetics, 142, 1491-1498. doi:10.1007/s00439-023-02597-3. https://pubmed.ncbi.nlm.nih.gov/37656279/
8. Xu, Xiaoqin, Yang, Xin, Liu, Xue, Cheng, Xiaolong, Xi, Yanfeng. . The Role of DBR1 as a Candidate Prognosis Biomarker in Esophageal Squamous Cell Carcinoma. In Technology in cancer research & treatment, 21, 15330338221083105. doi:10.1177/15330338221083105. https://pubmed.ncbi.nlm.nih.gov/35244467/
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