蛋白名称
Growth hormone receptor
免疫原
The antiserum was produced against synthesized peptide derived from the N-terminal region of human GHR. AA range:21-70
特异性
GHR Polyclonal Antibody detects endogenous levels of GHR protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Polyclonal, Rabbit,IgG
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
GHR; Growth hormone receptor; GH receptor; Somatotropin receptor
背景
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],
功能
disease:Defects in GHR are a cause of Laron dwarfism [MIM:262500]; also known as pituitary dwarfism II; Laron-type pituitary dwarfism I (LTD1) or Laron syndrome (LS). It is the most severe form of growth hormone insensitivity (GHI) characterized by growth impairment, dysmorphic facial features and truncal obesity. Levels of GHBP are low or undetectable in patients with Laron syndrome.,disease:Defects in GHR may be a cause of short stature [MIM:604271]. Short stature is defined by a subnormal rate of growth.,domain:The box 1 motif is required for JAK interaction and/or activation.,domain:The extracellular domain is the ligand-binding domain representing the growth hormone-binding protein (GHBP).,domain:The ubiquitination-dependent endocytosis motif (UbE) is required for recruitment of the ubiquitin conjugation system on to the receptor and for its internalization.,domain:The WSXWS motif a
