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Growth hormone receptor Recombinant Rabbit mAb (bsm-54199R)  
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產(chǎn)品編號(hào) bsm-54199R
英文名稱 Growth hormone receptor Recombinant Rabbit mAb
中文名稱 生長(zhǎng)激素受體抗體
別    名 GH receptor; GHBP; GHR; Growth hormone receptor precursor; Serum binding protein; Somatotropin receptor; GHR_HUMAN.  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  生長(zhǎng)因子和激素  細(xì)胞分化  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Recombinant
交叉反應(yīng) Human,Mouse (predicted: Rat)
產(chǎn)品應(yīng)用 WB=1:500,IHC-P=1:100-500,IHC-F=1:400-800,IF=1:100-500,ICC/IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 68 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Growth hormone receptor 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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. 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.

SWISS:
P10912

Gene ID:
2690

生長(zhǎng)激素受體(growth hormone receptor,GHR)是細(xì)胞因子/造血因子受體超級(jí)家族成員之一。
生長(zhǎng)激素受體通過(guò)二聚體的形式和生長(zhǎng)激素(growth hormone, GH)相結(jié)合,然后誘發(fā)Janus 激酶2 (Janus kinase 2,JAK2:是很多細(xì)胞因子、生長(zhǎng)因子及干擾素的重要信號(hào)傳感器)等細(xì)胞因子酪氨酸磷酸化并通過(guò)4條不同的途徑將信號(hào)傳入細(xì)胞內(nèi)從而產(chǎn)生一系列的生理效應(yīng)
產(chǎn)品圖片
Western blot analysis of Growth hormone receptor on different lysates with Rabbit anti-Growth hormone receptor antibody (bsm-54199R) at 1/500 dilution. Lane 1: Rat brain tissue lysate Lane 2: Mouse brain tissue lysate Lane 3: Human brain tissue lysate
Immunohistochemical analysis of paraffin-embedded mouse liver tissue using anti-Growth hormone receptor antibody. The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes.The tissues were blocked in 1% BSA for 30 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (bsm-54199R, 1/50) for 30 minutes at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
ICC staining of Growth hormone receptor in SH-SY5Y cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 10% negative goat serum for 15 minutes at room temperature. Cells were probed with the primary antibody (bsm-54199R, 1/200) for 1 hour at room temperature, washed with PBS. Alexa Fluor?488 conjugate-Goat anti-Rabbit IgG was used as the secondary antibody at 1/1,000 dilution. The nuclear counter stain is DAPI (blue).
ICC staining of Growth hormone receptor in JAR cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 10% negative goat serum for 15 minutes at room temperature. Cells were probed with the primary antibody (bsm-54199R, 1/200) for 1 hour at room temperature, washed with PBS. Alexa Fluor?488 conjugate-Goat anti-Rabbit IgG was used as the secondary antibody at 1/1,000 dilution. The nuclear counter stain is DAPI (blue).
ICC staining of Growth hormone receptor in HepG2 cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 10% negative goat serum for 15 minutes at room temperature. Cells were probed with the primary antibody (bsm-54199R, 1/200) for 1 hour at room temperature, washed with PBS. Alexa Fluor?488 conjugate-Goat anti-Rabbit IgG was used as the secondary antibody at 1/1,000 dilution. The nuclear counter stain is DAPI (blue).
ICC staining of Growth hormone receptor in A549 cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 10% negative goat serum for 15 minutes at room temperature. Cells were probed with the primary antibody (bsm-54199R, 1/200) for 1 hour at room temperature, washed with PBS. Alexa Fluor?488 conjugate-Goat anti-Rabbit IgG was used as the secondary antibody at 1/1,000 dilution. The nuclear counter stain is DAPI (blue).
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