C57BL/6JCya-Fbn1em1flox/Cya
Common Name:
Fbn1-flox
Product ID:
S-CKO-02369
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Fbn1-flox
Strain ID
CKOCMP-14118-Fbn1-B6J-VA
Gene Name
Product ID
S-CKO-02369
Gene Alias
B430209H23; Fib-1; Tsk
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
2
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Fbn1em1flox/Cya mice (Catalog S-CKO-02369) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000028633
NCBI RefSeq
NM_007993
Target Region
Exon 3
Size of Effective Region
~0.6 kb
Detailed Document
Overview of Gene Research
Fbn1 encodes the gene for fibrillin-1, a structural macromolecule that polymerizes into microfibrils present in all connective tissues and assembled into tissue-specific frameworks [1]. It is involved in regulating growth factor signaling. Mutations in Fbn1 are associated with various genetic disorders, highlighting its biological importance in connective tissue integrity and normal physiological function.
Fbn1 knockout in vascular smooth muscle cells in rats promotes cervical artery dissection. It leads to alternations in mRNA levels of extracellular matrix (ECM) proteins, decreased ECM protein levels and N-glycosylation abundance, affecting the stability of ECM, and thus the CAD process [2].
In conclusion, Fbn1 is crucial for the integrity of connective tissues by forming microfibrils. Model-based research, especially the Fbn1 knockout in rat VSMCs, reveals its role in maintaining ECM stability and its significance in preventing cervical artery dissection. Understanding Fbn1 is essential for elucidating the mechanisms of related genetic disorders.
References：
1. Sakai, Lynn Y, Keene, Douglas R, Renard, Marjolijn, De Backer, Julie. 2016. FBN1: The disease-causing gene for Marfan syndrome and other genetic disorders. In Gene, 591, 279-291. doi:10.1016/j.gene.2016.07.033. https://pubmed.ncbi.nlm.nih.gov/27437668/
2. Feng, Xiaochao, Zheng, Xixi, Lin, Aiqi, Wu, Weicheng, Han, Xiang. 2023. FBN1 knockout promotes cervical artery dissection by inducing N-glycosylation alternation of extracellular matrix proteins in rat VSMCs. In Cellular signalling, 110, 110834. doi:10.1016/j.cellsig.2023.110834. https://pubmed.ncbi.nlm.nih.gov/37532137/
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