C57BL/6JCya-Fbn2em1flox/Cya
Common Name
Fbn2-flox
Product ID
S-CKO-02370
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-14119-Fbn2-B6J-VA
Status
When using this mouse strain in a publication, please cite “Fbn2-flox Mouse (Catalog S-CKO-02370) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Fbn2-flox
Strain ID
CKOCMP-14119-Fbn2-B6J-VA
Gene Name
Product ID
S-CKO-02370
Gene Alias
sy, sne, Fib-2, mKIAA4226
Background
C57BL/6JCya
Gene Full Name
fibrillin 2
Modification
Conditional knockout
NCBI ID
Phenotype
Chromosome
Chr 18 (Mouse)
Application
--
Datasheet
Strain Description
Ensembl Transcript ID
ENSMUST00000025497
NCBI Transcript ID
NM_010181
Target Region
Exon 2
Size of Effective Region
~0.6 kb
Overview of Gene Research
Fbn2, encoding fibrillin-2, is associated with elastin fibres [2,4]. Its products play a role in the formation of the extracellular matrix, with implications for connective tissue integrity. The extracellular matrix organization pathway is one of the associated pathways [5].
Pathogenic variants in Fbn2 cause congenital contractural arachnodactyly (CCA), an autosomal dominant connective tissue disorder. CCA is characterized by features like arachnodactyly, camptodactyly, contracture of major joints, scoliosis, pectus deformities, and crumpled ears [1,3,6]. Some Fbn2 variants may also lead to severe cardiovascular or skeletal manifestations [1,3,5].
In bladder cancer, Fbn2 expression is upregulated and can be an independent prognostic factor, influencing the tumour microenvironment [2]. In hypoxic conditions, Fbn2 expression is reduced, inhibiting elastic fiber synthesis [4]. Also, Fbn2 promotes osteoblast proliferation, mineralization, and differentiation to accelerate fracture healing [7].
In conclusion, Fbn2 is crucial for connective tissue-related biological processes. Studies on Fbn2-related disorders such as CCA, and its role in bladder cancer, hypoxia-related elastic fiber impairment, and fracture healing, help us understand its biological functions. These insights may contribute to better management and treatment strategies for these conditions.
References：
1. Yang, Shulin, Li, Zongzhe. 2024. FBN2 pathogenic variants in congenital contractural arachnodactyly with severe cardiovascular manifestations. In Connective tissue research, 65, 214-225. doi:10.1080/03008207.2024.2340004. https://pubmed.ncbi.nlm.nih.gov/38602424/
2. Lu, Zechao, Lu, Zeguang, Lai, Yongchang, He, Zhaohui, Tang, Fucai. 2023. A comprehensive analysis of FBN2 in bladder cancer: A risk factor and the tumour microenvironment influencer. In IET systems biology, 17, 162-173. doi:10.1049/syb2.12067. https://pubmed.ncbi.nlm.nih.gov/37337404/
3. Huang, Yazhou, Fang, Xingxin, Ma, Linya, Gao, Taoran, Peng, Dan. 2025. FBN2 pathogenic mutation in congenital contractural arachnodactyly with severe skeletal manifestations. In Molecular genetics and metabolism reports, 42, 101193. doi:10.1016/j.ymgmr.2025.101193. https://pubmed.ncbi.nlm.nih.gov/39911746/
4. Boizot, Jérémy, Minville-Walz, Mélaine, Reinhardt, Dieter Peter, Sigaudo-Roussel, Dominique, Debret, Romain. 2022. FBN2 Silencing Recapitulates Hypoxic Conditions and Induces Elastic Fiber Impairment in Human Dermal Fibroblasts. In International journal of molecular sciences, 23, . doi:10.3390/ijms23031824. https://pubmed.ncbi.nlm.nih.gov/35163744/
5. Chen, Jing, Xiang, Qinqin, Xiao, Xiao, Yang, Mei, Liu, Shanling. 2022. Carrying both COL1A2 and FBN2 gene heterozygous mutations results in a severe skeletal clinical phenotype: an affected family. In BMC medical genomics, 15, 154. doi:10.1186/s12920-022-01296-8. https://pubmed.ncbi.nlm.nih.gov/35804365/
6. Sun, Liying, Huang, Yingzhao, Zhao, Sen, Tian, Wen, Wu, Nan. 2022. Identification of Novel FBN2 Variants in a Cohort of Congenital Contractural Arachnodactyly. In Frontiers in genetics, 13, 804202. doi:10.3389/fgene.2022.804202. https://pubmed.ncbi.nlm.nih.gov/35360850/
7. Huang, Jian, Huang, Jun, Li, Nan, Wang, Lanfang, Xiao, Quanhao. 2025. FBN2 promotes the proliferation, mineralization, and differentiation of osteoblasts to accelerate fracture healing. In Scientific reports, 15, 4843. doi:10.1038/s41598-025-89215-6. https://pubmed.ncbi.nlm.nih.gov/39924543/
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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