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C57BL/6JCya-Rmrpem1/Cya
Common Name:
Rmrp-KO
Product ID:
S-KO-20428
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Rmrp-KO
Strain ID
KOCMP-19782-Rmrp-B6J-VB
Gene Name
Rmrp
Product ID
S-KO-20428
Gene Alias
1110032O22Rik; Rmrpr
Background
C57BL/6JCya
NCBI ID
19782
Modification
Conventional knockout
Chromosome
4
Phenotype
MGI:97937
Document
Click here to download >>
Application
--
More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Rmrpem1/Cya mice (Catalog S-KO-20428) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000157463
NCBI RefSeq
NR_001460
Target Region
Exon 1
Size of Effective Region
~0.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Rmrp, the RNA component of mitochondrial RNA processing endoribonuclease, is a non-coding transcript. It has been associated with multiple biological processes and diseases. In normal biology, its exact function within the mitochondrial RNA processing endoribonuclease complex is crucial for proper cellular function. It has also been implicated in pathways related to cell proliferation, apoptosis, and differentiation, highlighting its overall biological importance. Genetic models, such as gene knockout (KO) or conditional knockout (CKO) mouse models, can potentially be used to further understand its functions [2,3].

Rmrp has been found to be involved in various diseases. In non-small cell lung cancer (NSCLC), m6A RNA methylation-mediated Rmrp stability promotes cell proliferation, invasion, and migration through regulating the TGFBR1/SMAD2/SMAD3 pathway [1]. In cartilage hair hypoplasia (CHH), biallelic pathogenic variants in Rmrp lead to this rare autosomal recessive condition, characterized by prenatal-onset growth failure, metaphyseal dysplasia, hair hypoplasia, immunodeficiency, etc. [3]. In the context of ligamentum flavum hypertrophy, Rmrp accelerates it by regulating GSDMD-mediated pyroptosis through Gli1 SUMOylation [4]. In Alzheimer's disease, Rmrp accelerates autophagy-mediated neurons apoptosis through the miR-3142/TRIB3 signaling axis [5]. In non-alcoholic fatty liver disease (NAFLD), Rmrp inhibition prevents disease progression via regulating the miR-206/PTPN1 axis [6]. In hepatocellular carcinoma, Rmrp knockdown suppresses cell proliferation, invasion, and migration by regulating miRNA-206/TACR1 [7]. In cardiomyocyte hypertrophy, linc-Rmrp regulates phenylephrine-induced hypertrophy by targeting miR-1 [8]. In osteoarthritis, Rmrp promotes chondrocyte injury by regulating the FOXC1/RBP4 axis [9]. In Schwann cells, Rmrp knockdown promotes proliferation and migration by mediating the miR-766-5p/CAND1 axis [10].

In conclusion, Rmrp plays essential roles in multiple biological processes and is closely associated with various diseases, including cancers, genetic disorders, neurodegenerative diseases, and more. The study of Rmrp using KO/CKO mouse models or other loss-of-function experiments could potentially provide valuable insights into disease mechanisms, which may offer new therapeutic strategies for these conditions.

References:
1. Yin, Hang, Chen, Lin, Piao, Shiqi, Zhang, Haiyang, Wang, Xiaoyuan. 2021. M6A RNA methylation-mediated RMRP stability renders proliferation and progression of non-small cell lung cancer through regulating TGFBR1/SMAD2/SMAD3 pathway. In Cell death and differentiation, 30, 605-617. doi:10.1038/s41418-021-00888-8. https://pubmed.ncbi.nlm.nih.gov/34628486/
2. Hussen, Bashdar Mahmud, Azimi, Tahereh, Hidayat, Hazha Jamal, Taheri, Mohammad, Ghafouri-Fard, Soudeh. 2021. Long Non-coding RNA RMRP in the Pathogenesis of Human Disorders. In Frontiers in cell and developmental biology, 9, 676588. doi:10.3389/fcell.2021.676588. https://pubmed.ncbi.nlm.nih.gov/33996836/
3. Uchida, Noboru, Ishii, Tomohiro, Nishimura, Gen, Tsujioka, Yuko, Hasegawa, Tomonobu. 2024. RMRP-related short stature: A report of six additional Japanese individuals with cartilage hair hypoplasia and literature review. In American journal of medical genetics. Part A, 194, e63562. doi:10.1002/ajmg.a.63562. https://pubmed.ncbi.nlm.nih.gov/38337186/
4. Yan, Xudong, Liu, Tinglong, Zhang, Run, Ma, Qinghong, Sun, Chao. 2024. RMRP accelerates ligamentum flavum hypertrophy by regulating GSDMD-mediated pyroptosis through Gli1 SUMOylation. In Frontiers in immunology, 15, 1427970. doi:10.3389/fimmu.2024.1427970. https://pubmed.ncbi.nlm.nih.gov/39221246/
5. Tang, Zhan-Bin, Chen, Hong-Ping, Zhong, Di, Yao, Jia-Lin, Li, Guo-Zhong. 2022. LncRNA RMRP accelerates autophagy-mediated neurons apoptosis through miR-3142/TRIB3 signaling axis in alzheimer's disease. In Brain research, 1785, 147884. doi:10.1016/j.brainres.2022.147884. https://pubmed.ncbi.nlm.nih.gov/35304105/
6. Yin, Jingjing, Chen, Xiangfei, Zhang, Fang, Zhao, Ming. 2022. RMRP inhibition prevents NAFLD progression in rats via regulating miR-206/PTPN1 axis. In Mammalian genome : official journal of the International Mammalian Genome Society, 33, 480-489. doi:10.1007/s00335-022-09945-0. https://pubmed.ncbi.nlm.nih.gov/35141790/
7. Hongfeng, Zhao, Andong, Ju, Liwen, Sun, Mingyong, Li, Aimin, Yue. 2019. lncRNA RMRP knockdown suppress hepatocellular carcinoma biological activities via regulation miRNA-206/TACR1. In Journal of cellular biochemistry, 121, 1690-1702. doi:10.1002/jcb.29404. https://pubmed.ncbi.nlm.nih.gov/31579977/
8. Chen, Jing, Li, Jia, Wang, Xuyan, Huang, Nina, Sun, Xiaohua. 2022. LincRNA RMRP Regulates Phenylephrine-induced Cardiomyocyte Hypertrophy by Means of Targeting miR-1. In Journal of cardiovascular pharmacology, 80, 709-717. doi:10.1097/FJC.0000000000001366. https://pubmed.ncbi.nlm.nih.gov/36070614/
9. Li, Jingyi, Zhou, Gang, Chen, Te, Lin, Qiao, Yang, Qiupeng. 2024. LncRNA RMRP promotes chondrocyte injury by regulating the FOXC1/RBP4 axis. In Central-European journal of immunology, 49, 366-382. doi:10.5114/ceji.2024.145312. https://pubmed.ncbi.nlm.nih.gov/39944260/
10. Zhou, Long, Yu, Xueyuan, Guo, Yuan, Liu, Xiangyu. 2021. LncRNA RMRP knockdown promotes proliferation and migration of Schwann cells by mediating the miR-766-5p/CAND1 axis. In Neuroscience letters, 770, 136440. doi:10.1016/j.neulet.2021.136440. https://pubmed.ncbi.nlm.nih.gov/34974108/
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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