C57BL/6JCya-Polrmtem1/Cya
Common Name:
Polrmt-KO
Product ID:
S-KO-17520
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Polrmt-KO
Strain ID
KOCMP-216151-Polrmt-B6J-VB
Gene Name
Product ID
S-KO-17520
Gene Alias
1110018N15Rik; 4932416K13; mtRPOL
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
10
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Polrmtem1/Cya mice (Catalog S-KO-17520) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000020580
NCBI RefSeq
NM_172551
Target Region
Exon 3
Size of Effective Region
~1.4 kb
Detailed Document
Overview of Gene Research
POLRMT, also known as mitochondrial RNA polymerase, is essential for the transcription of the mitochondrial genome. It plays a crucial role in mitochondrial biogenesis and functions related to the electron transport chain (ETC) as the mitochondrial genome encodes essential ETC proteins [2]. It is also involved in mitochondrial DNA replication, as demonstrated by the complete mtDNA loss when either TFB2M or POLRMT is knocked out in cultured human cybrid cells, indicating its importance in the priming of both strand-asynchronous and strand-coupled replication [6].
Mutations in POLRMT can lead to neurological diseases. Patients with POLRMT variants present with global developmental delay, hypotonia, short stature, and speech/intellectual disability in childhood, and one subject had progressive external ophthalmoplegia. Patient fibroblasts have a defect in mitochondrial mRNA synthesis. In vitro studies of recombinant POLRMT mutants show deleterious effects on mitochondrial transcription [1]. Additionally, targeting POLRMT has shown potential in cancer treatment. Inhibitors like IMT1 can disrupt mitochondrial functions in various cancer cells such as colorectal, prostate, and osteosarcoma cells, leading to decreased cell viability, proliferation, and migration, and inducing apoptosis. Genetic depletion of POLRMT also has similar effects, highlighting its importance in cancer cell growth [3,4,5].
In conclusion, POLRMT is vital for mitochondrial transcription and replication. Studies using loss-of-function models, such as gene knockout in cells, have revealed its role in neurological diseases and cancer. Targeting POLRMT could be a promising strategy for treating certain cancers, and understanding its function is crucial for uncovering disease mechanisms in both neurological and cancer-related conditions.
References:
1. Oláhová, Monika, Peter, Bradley, Szilagyi, Zsolt, Gustafsson, Claes M, Taylor, Robert W. 2021. POLRMT mutations impair mitochondrial transcription causing neurological disease. In Nature communications, 12, 1135. doi:10.1038/s41467-021-21279-0. https://pubmed.ncbi.nlm.nih.gov/33602924/
2. Yan, Weiwei, Xie, Chengmei, Sun, Sijun, Xu, Shuangnian, Wang, Yi-Ping. 2024. SUCLG1 restricts POLRMT succinylation to enhance mitochondrial biogenesis and leukemia progression. In The EMBO journal, 43, 2337-2367. doi:10.1038/s44318-024-00101-9. https://pubmed.ncbi.nlm.nih.gov/38649537/
3. Wang, Hao, Liu, Yuxin, Lu, Xing-Sheng, Gu, Wen, Yin, Guojian. 2024. Targeting POLRMT by IMT1 inhibits colorectal cancer cell growth. In Cell death & disease, 15, 643. doi:10.1038/s41419-024-07023-8. https://pubmed.ncbi.nlm.nih.gov/39227564/
4. Li, Xiaojun, Yao, Linya, Wang, Tao, Wu, Yufan, Jiang, Ting. 2023. Identification of the mitochondrial protein POLRMT as a potential therapeutic target of prostate cancer. In Cell death & disease, 14, 665. doi:10.1038/s41419-023-06203-2. https://pubmed.ncbi.nlm.nih.gov/37816734/
5. Kong, Yang, Li, Xiangrong, Zhang, Huanle, Yang, Hui-Lin, Dai, Jin. 2024. Targeting POLRMT by a first-in-class inhibitor IMT1 inhibits osteosarcoma cell growth in vitro and in vivo. In Cell death & disease, 15, 57. doi:10.1038/s41419-024-06444-9. https://pubmed.ncbi.nlm.nih.gov/38228583/
6. Inatomi, Teppei, Matsuda, Shigeru, Ishiuchi, Takashi, Yasukawa, Takehiro, Kang, Dongchon. 2021. TFB2M and POLRMT are essential for mammalian mitochondrial DNA replication. In Biochimica et biophysica acta. Molecular cell research, 1869, 119167. doi:10.1016/j.bbamcr.2021.119167. https://pubmed.ncbi.nlm.nih.gov/34744028/
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