生长**结合蛋白抗体
规格：1mg/1ml
英文名： Growth hormone-binding protein
别名： GH receptor; GHBP; GHR; Growth hormone receptor; Growth hormone receptor precursor; Serum binding protein; Somatotropin receptor; GHR_HUMAN.
分子量： 27/70kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Growth h
交叉反应：Human,
细胞定位：细胞膜
生长**结合蛋白抗体产品介绍：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生长**结合蛋白抗体 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生长**结合蛋白抗体 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. Gene ID: 2690 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
生长**结合蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:50-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid