C57BL/6JCya-Pcm1em1flox/Cya
Common Name:
Pcm1-flox
Product ID:
S-CKO-04182
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Pcm1-flox
Strain ID
CKOCMP-18536-Pcm1-B6J-VA
Gene Name
Product ID
S-CKO-04182
Gene Alias
2600002H09Rik; 9430077F19Rik; C030044G17Rik
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
8
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Pcm1em1flox/Cya mice (Catalog S-CKO-04182) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000045218
NCBI RefSeq
NM_023662
Target Region
Exon 6
Size of Effective Region
~0.7 kb
Detailed Document
Overview of Gene Research
PCM1, also known as pericentriolar material-1, is a protein that has multiple functions. It is a molecular marker of centriolar satellites, membrane-less organelles involved in numerous cellular and organismal processes [5]. In vertebrate radial glia progenitors, PCM1, asymmetrically distributed at the centrosomes, regulates polarized endosome dynamics and cell fate, facilitating endosome marker exchange and the formation of macromolecular complexes [3].
PCM1-JAK2 fusion is associated with various hematolymphoid neoplasms. In cases of myeloproliferative neoplasms, this fusion shows morphological similarities such as myeloproliferation, eosinophilia, and myelofibrosis, and clinical similarities like a striking male predominance and an aggressive course [4]. Patients with PCM1-JAK2 fusion-related neoplasia, including myeloproliferative neoplasms, acute leukemia, and T-cell cutaneous lymphoma, have different survival rates at 5-year follow-up. Recommended treatments include JAK2 inhibitors and hematologic stem cell transplantation, but the efficacy is limited by the small number of patients studied [2]. In a case of an atypical form of mycosis fungoides, the patient carried the isolated PCM1::JAK2 fusion and eosinophilia, with the disease being refractory to common treatments and demonstrating abnormal activation of the JAK-STAT pathway [1].
In conclusion, PCM1 has crucial functions in cellular processes such as regulating endosome dynamics and cell fate. The study of PCM1-JAK2 fusion in hematolymphoid neoplasms provides insights into disease mechanisms and potential treatment strategies, highlighting the importance of understanding PCM1-related genetic alterations in these diseases.
References:
1. Rodriguez-Sevilla, Juan Jose, Salido, Marta, Rodriguez-Rivera, Maria, Pujol, Ramon Maria, Colomo, Luis. 2022. PCM1::JAK2 fusion associates with an atypical form of mycosis fungoides. In Virchows Archiv : an international journal of pathology, 481, 967-973. doi:10.1007/s00428-022-03372-x. https://pubmed.ncbi.nlm.nih.gov/35786767/
2. Kaplan, Henry G, Jin, Ruyun, Bifulco, Carlo B, Scanlan, James M, Corwin, David R. . PCM1-JAK2 Fusion Tyrosine Kinase Gene-Related Neoplasia: A Systematic Review of the Clinical Literature. In The oncologist, 27, e661-e670. doi:10.1093/oncolo/oyac072. https://pubmed.ncbi.nlm.nih.gov/35472244/
3. Zhao, Xiang, Wang, Yiqi, Mouilleau, Vincent, Dong, Zhiqiang, Guo, Su. 2024. PCM1 conveys centrosome asymmetry to polarized endosome dynamics in regulating daughter cell fate. In bioRxiv : the preprint server for biology, , . doi:10.1101/2024.06.17.599416. https://pubmed.ncbi.nlm.nih.gov/38948739/
4. Hoeller, Sylvia, Walz, Christoph, Reiter, Andreas, Dirnhofer, Stephan, Tzankov, Alexandar. 2010. PCM1-JAK2-fusion: a potential treatment target in myelodysplastic-myeloproliferative and other hemato-lymphoid neoplasms. In Expert opinion on therapeutic targets, 15, 53-62. doi:10.1517/14728222.2011.538683. https://pubmed.ncbi.nlm.nih.gov/21091042/
5. Begar, Efe, Seyrek, Ece, Firat-Karalar, Elif Nur. 2024. Navigating centriolar satellites: the role of PCM1 in cellular and organismal processes. In The FEBS journal, 292, 688-708. doi:10.1111/febs.17194. https://pubmed.ncbi.nlm.nih.gov/38825736/
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