Datasheet

生物描述

特异性	FGFR3 Rabbit Recombinant mAb detects endogenous levels of total FGFR3.
背景	The signaling component of the mammalian Fibroblast Growth Factor (FGF) family is comprised of eighteen secreted proteins that interact with four signaling tyrosine kinase FGF receptors (FGFRs). Interaction of FGF ligands with their signaling receptors is regulated by protein or proteoglycan cofactors and by extracellular binding proteins. Activated FGFRs phosphorylate specific tyrosine residues that mediate interaction with cytosolic adaptor proteins and the RAS-MAPK, PI3K-AKT, PLCγ, and STAT intracellular signaling pathways. Gain-of-function missense mutations in FGFR3 causes autosomal dominant disorders such as Hypochondroplasia, Achondroplasia, Thanatophoric dysplasia, and Platyspondylic lethal skeletal dysplasia. Loss-of-function missense mutations that likely function through a dominant negative mechanism in FGFR3 causes CATSHL syndrome (autosomal dominant syndrome characterized by camptodactyly, tall stature, and hearing loss).

特异性

FGFR3 Rabbit Recombinant mAb detects endogenous levels of total FGFR3.

背景

The signaling component of the mammalian Fibroblast Growth Factor (FGF) family is comprised of eighteen secreted proteins that interact with four signaling tyrosine kinase FGF receptors (FGFRs). Interaction of FGF ligands with their signaling receptors is regulated by protein or proteoglycan cofactors and by extracellular binding proteins. Activated FGFRs phosphorylate specific tyrosine residues that mediate interaction with cytosolic adaptor proteins and the RAS-MAPK, PI3K-AKT, PLCγ, and STAT intracellular signaling pathways. Gain-of-function missense mutations in FGFR3 causes autosomal dominant disorders such as Hypochondroplasia, Achondroplasia, Thanatophoric dysplasia, and Platyspondylic lethal skeletal dysplasia. Loss-of-function missense mutations that likely function through a dominant negative mechanism in FGFR3 causes CATSHL syndrome (autosomal dominant syndrome characterized by camptodactyly, tall stature, and hearing loss).

使用信息

抗体应用

wb,ELISA,ELISA

稀释比例

WB

1:1000

反应性

Human Mouse Rat

MW (kDa)

98kDa

抗体类型

储存液配方

10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide.

储存条件
(自收到货起)

Store at –20°C.

文献参考

[1] David M Ornitz, et al. Wiley Interdiscip Rev Dev Biol. 2015, 4(3): 215–266.

Application Data

WB

Validated by Selleck