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C57BL/6JCya-Osgepem1/Cya
Common Name:
Osgep-KO
Product ID:
S-KO-11610
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
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Basic Information
Strain Name
Osgep-KO
Strain ID
KOCMP-66246-Osgep-B6J-VB
Gene Name
Osgep
Product ID
S-KO-11610
Gene Alias
1500019L24Rik; GCPL-1; PRSMG1
Background
C57BL/6JCya
NCBI ID
66246
Modification
Conventional knockout
Chromosome
14
Phenotype
MGI:1913496
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-Osgepem1/Cya mice (Catalog S-KO-11610) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000159292
NCBI RefSeq
NM_133676.2
Target Region
Exon 2~3
Size of Effective Region
~1.0 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Osgep, also known as O-sialoglycoprotein endopeptidase, is a crucial element of the highly conserved KEOPS complex. It is responsible for t6A37 modification of tRNANNU, which tunes glucose metabolism, and also plays a role in processes like protein translation, endoplasmic reticulum (ER) stress response, and cell proliferation [1,2,4]. The KEOPS complex, of which Osgep is a part, catalyzes an essential posttranscriptional modification of tRNA, highlighting its overall biological importance [4]. Genetic models such as knockout (KO) or conditional knockout (CKO) mouse models are valuable for studying Osgep.

Global Osgep deletion in mice causes glucose intolerance, while β-cell-specific deletion leads to hyperglycemia and glucose intolerance due to impaired insulin activity. Transcriptomics and proteomics in Osgep-deficient islets show activation of the unfolded protein response (UPR) and apoptosis signaling pathways, linked to misfolded proinsulin from reduced t6A37 modification. Overexpression of Osgep in the pancreas rescues insulin secretion and mitigates diabetes in high-fat diet mice [1]. In zebrafish and mice, CRISPR-Cas9 knockout of genes in the KEOPS complex, including Osgep, recapitulates the human phenotype of primary microcephaly and results in early lethality. Knockdown of Osgep in cell models inhibits cell proliferation, impairs protein translation, causes ER stress, activates DNA-damage-response signaling, and ultimately induces apoptosis [2].

In conclusion, Osgep is essential for maintaining proper islet β-cell function, glucose homeostasis, and normal development. Studies using KO/CKO mouse models have revealed its role in diabetes-related processes and in Galloway-Mowat syndrome, which is characterized by early-onset nephrotic syndrome and microcephaly with brain anomalies. Understanding Osgep's functions through these model-based research provides insights into potential therapeutic targets for diabetes and may help in early diagnosis and genetic counseling for Galloway-Mowat syndrome [1,2,3,5,6].

References:
1. Liu, Yujie, Yang, Xuechun, Zhou, Jian, Zhou, Honghao, Li, Qing. 2024. OSGEP regulates islet β-cell function by modulating proinsulin translation and maintaining ER stress homeostasis in mice. In Nature communications, 15, 10479. doi:10.1038/s41467-024-54905-8. https://pubmed.ncbi.nlm.nih.gov/39622811/
2. Braun, Daniela A, Rao, Jia, Mollet, Geraldine, Antignac, Corinne, Hildebrandt, Friedhelm. 2017. Mutations in KEOPS-complex genes cause nephrotic syndrome with primary microcephaly. In Nature genetics, 49, 1529-1538. doi:10.1038/ng.3933. https://pubmed.ncbi.nlm.nih.gov/28805828/
3. Domingo-Gallego, Andrea, Furlano, Mónica, Pybus, Marc, Torra, Roser, Ars, Elisabet. 2019. Novel homozygous OSGEP gene pathogenic variants in two unrelated patients with Galloway-Mowat syndrome: case report and review of the literature. In BMC nephrology, 20, 126. doi:10.1186/s12882-019-1317-y. https://pubmed.ncbi.nlm.nih.gov/30975089/
4. Krausel, Vanessa, Pund, Lisanne, Nüsse, Harald, Krahn, Michael P, Braun, Daniela A. 2022. The transcription factor ATF4 mediates endoplasmic reticulum stress-related podocyte injury and slit diaphragm defects. In Kidney international, 103, 872-885. doi:10.1016/j.kint.2022.11.024. https://pubmed.ncbi.nlm.nih.gov/36587794/
5. Xu, Suhua, Hu, Lan, Yang, Lin, Zhang, Peng, Hu, Liyuan. 2022. Galloway-Mowat Syndrome Type 3 Caused by OSGEP Gene Variants: A Case Report and Literature Review. In Frontiers in pediatrics, 10, 899991. doi:10.3389/fped.2022.899991. https://pubmed.ncbi.nlm.nih.gov/35783322/
6. Lin, Pei-Yi, Tseng, Min-Hua, Zenker, Martin, Lin, Shuan-Pei, Tsai, Jeng-Daw. 2018. Galloway-Mowat syndrome in Taiwan: OSGEP mutation and unique clinical phenotype. In Orphanet journal of rare diseases, 13, 226. doi:10.1186/s13023-018-0961-9. https://pubmed.ncbi.nlm.nih.gov/30558655/
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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