C57BL/6JCya-Ppm1kem1flox/Cya
Common Name:
Ppm1k-flox
Product ID:
S-CKO-08516
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Ppm1k-flox
Strain ID
CKOCMP-243382-Ppm1k-B6J-VA
Gene Name
Product ID
S-CKO-08516
Gene Alias
2900063A19Rik; A930026L03Rik; PP2C-kappa; PP2Cm
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
6
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ppm1kem1flox/Cya mice (Catalog S-CKO-08516) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000042766
NCBI RefSeq
NM_175523
Target Region
Exon 3
Size of Effective Region
~0.6 kb
Detailed Document
Overview of Gene Research
Ppm1k, also named as PP2Cm or branched-chain α -ketoacid dehydrogenase complex phosphatase, is a member of the metal-dependent phosphatase family. It is predominantly located in the mitochondrial matrix and plays a crucial role in the metabolism of branched-chain amino acids (BCAAs). Ppm1k is associated with various cellular functions and is related to diseases such as Alzheimer's, cardiomyopathy, and maple syrup urine disease (MSUD) [3,4].
Ppm1k-deficient female mice had increased BCAA levels and exhibited PCOS-like traits, including hyperandrogenemia and abnormal follicle development. A reduction in dietary BCAA intake significantly improved the endocrine and ovarian dysfunction of Ppm1k-/-female mice. In human granulosa cells, knockdown of PPM1K promoted the conversion of glycolysis to pentose phosphate pathway and inhibited mitochondrial oxidative phosphorylation, indicating that Ppm1k deficiency-impaired BCAA catabolism causes the occurrence and development of PCOS [1]. Also, in neurons, knockdown of PPM1K induced neuronal ferroptosis due to defective BCAA catabolism after cerebral ischemia-reperfusion injury [2].
In conclusion, Ppm1k is essential for the normal catabolism of BCAAs. The study of Ppm1k-deficient mouse models has revealed its significance in diseases like PCOS and cerebral ischemia-reperfusion injury, highlighting its potential as a therapeutic target in these disease areas.
References：
1. Mu, Liangshan, Ye, Zhenhong, Hu, Junhao, Zhao, Yue, Qiao, Jie. 2023. PPM1K-regulated impaired catabolism of branched-chain amino acids orchestrates polycystic ovary syndrome. In EBioMedicine, 89, 104492. doi:10.1016/j.ebiom.2023.104492. https://pubmed.ncbi.nlm.nih.gov/36863088/
2. Li, Tao, Zhao, Lili, Li, Ye, Kuang, Fang, Zhang, Guilian. 2023. PPM1K mediates metabolic disorder of branched-chain amino acid and regulates cerebral ischemia-reperfusion injury by activating ferroptosis in neurons. In Cell death & disease, 14, 634. doi:10.1038/s41419-023-06135-x. https://pubmed.ncbi.nlm.nih.gov/37752100/
3. Kamada, Rui, Kudoh, Fuki, Ito, Shogo, Omichinski, James G, Sakaguchi, Kazuyasu. 2020. Metal-dependent Ser/Thr protein phosphatase PPM family: Evolution, structures, diseases and inhibitors. In Pharmacology & therapeutics, 215, 107622. doi:10.1016/j.pharmthera.2020.107622. https://pubmed.ncbi.nlm.nih.gov/32650009/
4. Mao, Yuanling, Feng, Jing. 2024. Phosphatase activity-based PPM1K: a key player in the regulation of mitochondrial function and its multifaceted impact in diseases. In Molecular and cellular biochemistry, 480, 2815-2826. doi:10.1007/s11010-024-05188-6. https://pubmed.ncbi.nlm.nih.gov/39695034/
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