B6-huFGFR1 Mouse
Product Name
B6-huFGFR1 Mouse
Product ID
C001900
Strain Name
C57BL/6NCya-Fgfr1tm1(hFGFR1)/Cya
Backgroud
C57BL/6NCya
Status
When using this mouse strain in a publication, please cite “B6-huFGFR1 Mouse (Catalog C001900) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
Gene Alias
CEK, FLG, HH2, OGD, ECCL, FLT2, KAL2, BFGFR, CD331, FGFBR, FLT-2, HBGFR, N-SAM, FGFR-1, HRTFDS, bFGF-R-1
NCBI ID
Chromosome
Chr 8
MGI ID
Datasheet
Strain Description
The FGFR1 gene provides instructions for the synthesis of the fibroblast growth factor receptor 1, a member of the receptor tyrosine kinase (RTK) family. This protein is characterized by an extracellular region with three immunoglobulin-like domains for ligand binding, a single transmembrane segment, and an intracellular tyrosine kinase domain that triggers downstream signaling cascades like the MAPK/ERK and PI3K/AKT pathways. FGFR1 is widely expressed across diverse tissues, with particularly high levels in the developing mesoderm, skeletal system, and the central nervous system, where it is essential for the migration of gonadotropin-releasing hormone (GnRH) neurons and olfactory bulb development [1]. Functionally, it acts as a master regulator of cell proliferation, differentiation, and survival, playing a pivotal role in embryonic limb induction and adult tissue homeostasis [2]. Mutations or chromosomal aberrations in FGFR1 are linked to a diverse array of diseases: gain-of-function mutations cause craniosynostosis syndromes like Pfeiffer and Jackson-Weiss syndromes, while loss-of-function variants lead to Kallmann syndrome (characterized by delayed puberty and an absent sense of smell) [3]. Additionally, FGFR1 gene amplifications and rearrangements are significant oncogenic drivers in various cancers, including squamous cell lung cancer, certain breast cancers, and 8p11 myeloproliferative syndrome [4].
The B6-huFGFR1 mouse is a humanized model constructed through gene-editing technology, in which the mouse Fgfr1 endogenous extracellular domain genomic DNA is replaced with the human FGFR1 extracellular domain genomic DNA. This model can be used for research on craniosynostosis syndromes, Kallmann syndrome, and various cancers, as well as for screening, development, and preclinical evaluation of FGFR1-targeted therapeutics.
Reference
Yamaguchi TP, Conlon RA, Rossant J. Expression of the fibroblast growth factor receptor FGFR-1/flg during gastrulation and segmentation in the mouse embryo. Dev Biol. 1992 Jul;152(1):75-88.
Li C, Xu X, Nelson DK, Williams T, Kuehn MR, Deng CX. FGFR1 function at the earliest stages of mouse limb development plays an indispensable role in subsequent autopod morphogenesis. Development. 2005 Nov;132(21):4755-64.
Soejima Y, Otsuka Y, Kawaguchi M, Oguni K, Yamamoto K, Nakano Y, Yasuda M, Tokumasu K, Ueda K, Hasegawa K, Iwata N, Otsuka F. Involvement of a Novel Variant of FGFR1 Detected in an Adult Patient with Kallmann Syndrome in Regulation of Gonadal Steroidogenesis. Int J Mol Sci. 2025 Mar 18;26(6):2713.
Hong SH, Kim TM. High-resolution genomic configuration of FGFR rearrangements dictates the therapeutic vulnerability of squamous cell lung cancers. Transl Lung Cancer Res. 2024 Feb 29;13(2):236-239.
Strain Strategy
The mouse Fgfr1 endogenous extracellular domain genomic DNA was replaced with the human FGFR1 extracellular domain genomic DNA. The expression of mouse Gm16159 may be affected by the deletion of this KO region.
Figure 1. Diagram of the gene editing strategy for the generation of B6-huFGFR1 mice.
Application Area
Screening, development, and pre-clinical evaluation of FGFR1-targeted drugs;
Research on the pathogenic mechanisms and relevant treatment methods of craniosynostosis syndromes like Pfeiffer and Jackson-Weiss syndromes;
Research on the pathogenic mechanism and relevant treatment methods of Kallmann syndrome;
Research on the pathogenic mechanisms and relevant treatment methods of various cancers such as squamous cell lung cancer, certain breast cancers, and 8p11 myeloproliferative syndrome.
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