C57BL/6JCya-Pdgfrbem1/Cya
Common Name:
Pdgfrb-KO
Product ID:
S-KO-17901
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Pdgfrb-KO
Strain ID
KOCMP-18596-Pdgfrb-B6J-VB
Gene Name
Product ID
S-KO-17901
Gene Alias
CD140b; PDGFR-1; Pdgfr
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
18
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pdgfrbem1/Cya mice (Catalog S-KO-17901) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000025522
NCBI RefSeq
NM_001146268
Target Region
Exon 3~15
Size of Effective Region
~16.2 kb
Detailed Document
Overview of Gene Research
Pdgfrb, encoding the platelet-derived growth factor receptor-beta, is expressed in neurons, vascular smooth muscle cells, and pericytes [4]. It likely plays crucial roles in cell growth, differentiation, and migration, and is involved in multiple cellular pathways, though specific pathways are not elaborated in the provided abstracts. Genetic models are valuable for studying its functions.
In intracranial fusiform aneurysms, whole-exome sequencing detected PDGFRB gene mutations, and subsequent experiments on mutant smooth muscle cell lines and zebrafish models showed that PDGFRB somatic variants promoted smooth muscle cell phenotype modulation. The JAK-STAT pathway played a crucial role in this process, and the JAK inhibitor Ruxolitinib could reverse the phenotype modulation and inhibit vascular anomalies induced by PDGFRB mutation [1]. In pericytic tumors, PDGFRB mutations were detected in various types including myopericytomas, angioleiomyomas, and glomus tumors, but these mutations were not useful for subclassifying these tumors [2]. A patient with myeloid neoplasm having a PCM1-PDGFRB fusion achieved complete molecular remission after low-dose imatinib treatment [3]. A novel PDGFRB sequence variant was found in a family with a mild form of primary familial brain calcification [4].
In conclusion, Pdgfrb is involved in important biological processes related to cell phenotypes in diseases such as intracranial aneurysms and pericytic tumors. Model-based research, like that on zebrafish and cell lines in intracranial aneurysm studies, has revealed its role in smooth muscle cell phenotype modulation, providing insights into potential therapeutic targets for such diseases.
References:
1. Hao, Li, Ya, Xiaolong, Wu, Jiaye, Lin, Qing, Zhao, Jizong. 2024. Somatic PDGFRB activating variants promote smooth muscle cell phenotype modulation in intracranial fusiform aneurysm. In Journal of biomedical science, 31, 51. doi:10.1186/s12929-024-01040-7. https://pubmed.ncbi.nlm.nih.gov/38741091/
2. Iwamura, Ryuji, Komatsu, Kazuki, Kusano, Midori, Itami, Hiroe, Hisaoka, Masanori. 2023. PDGFRB and NOTCH3 Mutations are Detectable in a Wider Range of Pericytic Tumors, Including Myopericytomas, Angioleiomyomas, Glomus Tumors, and Their Combined Tumors. In Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 36, 100070. doi:10.1016/j.modpat.2022.100070. https://pubmed.ncbi.nlm.nih.gov/36788105/
3. Wang, Zhe, Wan, Li, Lin, Dong, Tian, Zheng, Mi, Ying-Chang. 2022. Myeloid Neoplasm with PCM1-PDGFRB Transcript Responded to Low-Dose Imatinib: One Case Report with Literature Review. In Acta haematologica, 145, 560-565. doi:10.1159/000524275. https://pubmed.ncbi.nlm.nih.gov/35340014/
4. Mathorne, Stine Westergaard, Sørensen, Kristina, Fagerberg, Christina, Bode, Matthias, Hertz, Jens Michael. 2019. A novel PDGFRB sequence variant in a family with a mild form of primary familial brain calcification: a case report and a review of the literature. In BMC neurology, 19, 60. doi:10.1186/s12883-019-1292-8. https://pubmed.ncbi.nlm.nih.gov/30979360/
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