生长激素受体(growth hormone receptor，GHR)是细胞因子/造血因子受体超级家族成员之一。
生长激素受体通过二聚体的形式和生长激素(growth hormone, GH)相结合，然后诱发Janus 激酶2 (Janus kinase 2，JAK2：是很多细胞因子、生长因子及干扰素的重要信号传感器)等细胞因子酪氨酸磷酸化并通过4条不同的途径将信号传入细胞内从而产生一系列的生理效应

背景资料

This gene encodes a member of the type I cytokine receptor family, which is a transmembrane receptor for growth hormone. Binding of growth hormone to the receptor leads to receptor dimerization and the activation of an intra- and intercellular signal transduction pathway leading to growth. Mutations in this gene have been associated with Laron syndrome, also known as the growth hormone insensitivity syndrome (GHIS), a disorder characterized by short stature. In humans and rabbits, but not rodents, growth hormone binding protein (GHBP) is generated by proteolytic cleavage of the extracellular ligand-binding domain from the mature growth hormone receptor protein. Multiple alternatively spliced transcript variants have been found for this gene.[provided by RefSeq, Jun 2011].

产品应用

应用	已检合格种属	预测种属	推荐稀释比例
WB	Mouse, Rat	Human, Pig, Sheep, Cow, Chicken, Dog	1:500-2000
IHC-P	Mouse, Rat	Human, Pig, Sheep, Cow, Chicken, Dog	1:100-500
IHC-F	Mouse, Rat	Human, Pig, Sheep, Cow, Chicken, Dog	1:100-500
IF	Mouse, Rat	Human, Pig, Sheep, Cow, Chicken, Dog	1:100-500
Flow-Cyt	Human	Mouse, Rat, Pig, Sheep, Cow, Chicken, Dog	1μg/Test

交叉反应

交叉反应: Human, Mouse, Rat (predicted: Pig, Sheep, Cow, Chicken, Dog)

靶标

基因名

GHR

蛋白名

Growth hormone receptor

亚基

On growth hormone (GH) binding, forms homodimers and binds JAK2 via a box 1-containing domain. Binding to SOCS3 inhibits JAK2 activation, binding to CIS and SOCS2 inhibits STAT5 activation. Interacts with ADAM17.

亚细胞定位

Cell membrane; Single-pass type I membrane protein. Note=On growth hormone binding, GHR is ubiquitinated, internalized, down-regulated and transported into a degradative or non-degradative pathway.
Isoform 2: Cell membrane; Single-pass type I membrane protein. Note=Remains fixed to the cell membrane and is not internalized.
Growth hormone-binding protein: Secreted. Note=Complexed to a substantial fraction of circulating GH.

组织特异性

Expressed in various tissues with high expression in liver and skeletal muscle. Isoform 4 is predominantly expressed in kidney, bladder, adrenal gland and brain stem. Isoform 1 expression in placenta is predominant in chorion and decidua. Isoform 4 is highly expressed in placental villi. Isoform 2 is expressed in lung, stomach and muscle. Low levels in liver.

翻译后修饰

The soluble form (GHBP) is produced by phorbol ester-promoted proteolytic cleavage at the cell surface (shedding) by ADAM17/TACE. Shedding is inhibited by growth hormone (GH) binding to the receptor probably due to a conformational change in GHR rendering the receptor inaccessible to ADAM17.
On GH binding, phosphorylated on tyrosine residues in the cytoplasmic domain by JAK2.
On ligand binding, ubiquitinated on lysine residues in the cytoplasmic domain. This ubiquitination is not sufficient for GHR internalization.

疾病

Laron syndrome (LARS) [MIM:262500]: A severe form of growth hormone insensitivity characterized by growth impairment, short stature, dysfunctional growth hormone receptor, and failure to generate insulin-like growth factor I in response to growth hormone. Note=The disease is caused by mutations affecting the gene represented in this entry.
Idiopathic short stature autosomal (ISSA) [MIM:604271]: Short stature is defined by a subnormal rate of growth. Note=The disease is caused by mutations affecting the gene represented in this entry.

相似性

Belongs to the type I cytokine receptor family. Type 1 subfamily.
Contains 1 fibronectin type-III domain.

功能

Receptor for pituitary gland growth hormone involved in regulating postnatal body growth. On ligand binding, couples to the JAK2/STAT5 pathway.
The soluble form (GHBP) acts as a reservoir of growth hormone in plasma and may be a modulator/inhibitor of GH signaling.
Isoform 2 up-regulates the production of GHBP and acts as a negative inhibitor of GH signaling.

Growth hormone receptor Rabbit pAb (一抗) | Bioss

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