产品名称：Anti-GHR/FITC抗体

Anti-GHR/FITC抗体

产品编号YB-0654R-FITC

英文名称Anti-GHR/FITC

中文名称FITC标记的生长**受体抗体

别    名GH receptor; GHBP; GHR; Growth hormone receptor; Growth hormone receptor precursor; Serum binding protein; Somatotropin receptor; GHR_HUMAN.  

Anti-GHR/FITC抗体

说 明 书100ul  

研究领域细胞生物  发育生物学  信号转导  生长因子和**  细胞分化  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,

产品应用Flow-Cyt=1:50-200 IF=1:50-200  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量68kDa

Anti-GHR/FITC抗体性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human GHR

亚    型IgG

纯化方法affinity purified by Protein A

储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件Store at -20 癈 for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20癈. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 癈.

Anti-GHR/FITC抗体

产品介绍background:

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].

Function:

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.

Subunit:

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.

Subcellular Location:

Cell membrane; Single-pass type I membrane protein.Anti-GHR/FITC抗体 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.

Tissue Specificity:

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.

Post-translational modifications:

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.

DISEASE:

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.

Similarity:

Belongs to the type I cytokine receptor family. Type 1 subfamily.

Contains 1 fibronectin type-III domain.

Database links:

Entrez Gene: 2690 Human

Entrez Gene: 14600 Mouse

Entrez Gene: 25235 Rat

Omim: 600946 Human

SwissProt: P10912 Human

SwissProt: P16882 Mouse

SwissProt: P16310 Rat

Unigene: 125180 Human

Unigene: 684631 Human

Unigene: 3986 Mouse

Unigene: 2178 Rat

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

生长**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**受体通过二聚体的形式和生长**(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。