| 產(chǎn)品編號(hào) | bs-0654R-BF647 |
| 英文名稱1 | Rabbit Anti-Growth hormone receptor/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的生長(zhǎng)激素受體抗體 |
| 別 名 | GH receptor; GHBP; GHR; Growth hormone receptor precursor; Serum binding protein; Somatotropin receptor; GHR_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買(mǎi) 大包裝/詢價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 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 |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, Mouse, Rat, (predicted: Chicken, Dog, Pig, Cow, Sheep, ) |
| 產(chǎn)品應(yīng)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 |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GHR |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C 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°C. 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 °C. |
| 產(chǎn)品介紹 |
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. 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 Omim: 600946 Human SwissProt: P10912 Human SwissProt: P16882 Mouse 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)。 |
