B6-hFBN1
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Model Name
B6-hFBN1
Product ID
C001733
Gene Name
Background
C57BL/6JCya
Gene Alias
FBN; SGS; WMS; MASS; MFLS; MFS1; OCTD; SSKS; WMS2; ACMICD; ECTOL1; GPHYSD2
Classification
Full-Gene Humanized Models,Rare Disease Models
NCBI ID
Phenotype
Chromosome
15
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Note: When using this mouse strain in a publication, please cite “B6-hFBN1 mice (Catalog C001733) were purchased from Cyagen.”
Strain Description
The B6-hFBN1 mouse is a humanized model, generated by in situ replacement of the mouse Fbn1 gene sequence (including 3'UTR) with the corresponding human FBN1 sequence while retaining the mouse signal peptide. This model is suitable for research on the pathogenesis and therapeutic agents for Marfan syndrome (MFS), dominant Weill-Marchesani syndrome, scleroderma, and other related disorders. Additionally, leveraging its proprietary TurboKnockout fusion BAC recombination technology, Cyagen can provide popular mutation disease models based on this platform or offer customized services for different mutations to meet the experimental needs of researchers.
Application Area
Research on Marfan syndrome (MFS)
Research on the pathogenesis and therapeutic agents for dominant Weill-Marchesani syndrome, scleroderma, and other related disorders
Validation Data