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C57BL/6JCya-Hes7em1/Cya
Common Name:
Hes7-KO
Product ID:
S-KO-19112
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Hes7-KO
Strain ID
KOCMP-84653-Hes7-B6J-VA
Gene Name
Hes7
Product ID
S-KO-19112
Gene Alias
bHLHb37
Background
C57BL/6JCya
NCBI ID
84653
Modification
Conventional knockout
Chromosome
11
Phenotype
MGI:2135679
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Hes7em1/Cya mice (Catalog S-KO-19112) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000024543
NCBI RefSeq
NM_033041
Target Region
Exon 2~4
Size of Effective Region
~1.8 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Hes7, a bHLH-type repressor gene, is crucial for somitogenesis. It is regulated by Notch signaling and expressed in the presomitic mesoderm. Its oscillatory expression controls the periodic segmentation of the presomitic mesoderm into somites, which form the vertebral column and associated tissues. Hes7 is also involved in Fgf and Notch signaling pathways, which are important for regulating the timing of somite formation [2,4].

In mouse models, various studies have demonstrated the significance of Hes7. Mutant mice expressing Hes7 with a longer half-life showed severely disorganized somite segmentation and oscillatory expression after a few normal cycles, indicating that the instability of Hes7 is essential for sustained oscillation and its function as a segmentation clock [6]. Inserting a large intron into Hes7 3'UTR led to loss of 3'UTR, reduction of Hes7 protein, damped oscillation, and improper periodic somite segmentation, showing that 3'UTR is essential for accumulating adequate Hes7 protein for the somite segmentation clock [5]. Additionally, Fgf4 mutants, which display vertebral defects, were found to have abnormal Hes7 levels, suggesting Fgf4 maintains Hes7 levels critical for normal somite segmentation clock function [3]. In humans, homozygous or compound heterozygous HES7 variants can cause spondylocostal dysostosis 4 (SCDO4), characterized by short stature, dyspnea, and spinal deformities [1].

In conclusion, Hes7 is essential for the proper formation of somites during vertebrate development. Mouse models have been instrumental in revealing the molecular mechanisms underlying its function, such as the importance of protein instability and 3'UTR for its role in the segmentation clock. In humans, HES7 variants are associated with SCDO4, highlighting the translational relevance of Hes7 research in understanding congenital spine diseases.

References:
1. Lv, Shaoguang, Wu, Yuanyuan, Liu, Fang, Jiao, Baoquan. 2023. A novel homozygous HES7 splicing variant causing spondylocostal dysostosis 4: a case report. In Frontiers in pediatrics, 11, 1201999. doi:10.3389/fped.2023.1201999. https://pubmed.ncbi.nlm.nih.gov/37691774/
2. Harima, Yukiko, Kageyama, Ryoichiro. 2013. Oscillatory links of Fgf signaling and Hes7 in the segmentation clock. In Current opinion in genetics & development, 23, 484-90. doi:10.1016/j.gde.2013.02.005. https://pubmed.ncbi.nlm.nih.gov/23465881/
3. Anderson, Matthew J, Magidson, Valentin, Kageyama, Ryoichiro, Lewandoski, Mark. 2020. Fgf4 maintains Hes7 levels critical for normal somite segmentation clock function. In eLife, 9, . doi:10.7554/eLife.55608. https://pubmed.ncbi.nlm.nih.gov/33210601/
4. Bessho, Y, Miyoshi, G, Sakata, R, Kageyama, R. . Hes7: a bHLH-type repressor gene regulated by Notch and expressed in the presomitic mesoderm. In Genes to cells : devoted to molecular & cellular mechanisms, 6, 175-85. doi:. https://pubmed.ncbi.nlm.nih.gov/11260262/
5. Fujimuro, Takeshi, Matsui, Takaaki, Nitanda, Yasuhide, Nakahata, Yasukazu, Bessho, Yasumasa. 2014. Hes7 3'UTR is required for somite segmentation function. In Scientific reports, 4, 6462. doi:10.1038/srep06462. https://pubmed.ncbi.nlm.nih.gov/25248974/
6. Hirata, Hiromi, Bessho, Yasumasa, Kokubu, Hiroshi, Lewis, Julian, Kageyama, Ryoichiro. 2004. Instability of Hes7 protein is crucial for the somite segmentation clock. In Nature genetics, 36, 750-4. doi:. https://pubmed.ncbi.nlm.nih.gov/15170214/
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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