G蛋白αS抗体
规格：1mg/1ml
英文名： G protein alpha S/NESP55
别名： Adenylate cyclase stimulating G alpha protein; AHO; Alternative gene product encoded by XL exon; Extra large alphas protein; GNAS; GNAS complex locus; GNAS1; GPSA; Gs alpha subunit; GSA; GSP; Guanine
分子量： 111kDa
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 G protei
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, Danio rerio
细胞定位：细胞膜
G蛋白αS抗体产品介绍：background: Guanine nucleotide-binding proteins (G proteins) are involved as modulators or transducers in various transmembrane signaling systems. The Gs protein is involved in hormonal regulation of adenylate cyclase: it activates the cyclase in response to beta-adrenergic stimuli. Function: Guanine nucleotide-binding proteins (G proteins) are involved as modulators or transducers in various transmembrane signaling systems. The G(s) protein is involved in hormonal regulation of adenylate cyclase: it activates the cyclase in response to beta-adrenergic stimuli. XLas isoforms interact with the same set of receptors as Gnas isoforms (By similarity). Subunit: G proteins are composed of 3 units; alpha, beta and gamma. The alpha chain contains the guanine nucleotide binding site. Interacts through its N-terminal region with ALEX which is produced from the same locus in a different open reading frame. This interaction may inhibit its adenylyl cyclase-stimulating activity (By similarity). Subcellular Location: Cell membrane; Peripheral membrane protein. DISEASE: Defects in GNAS are the cause of GNAS hyperfunction (GNASHYP) [MIM:139320]. This condition is characterized by increased trauma-related bleeding tendency, prolonged bleeding time, brachydactyly and mental retardation. Both the XLas isoforms and the ALEX protein are mutated which strongly reduces the interaction between them and this may allow unimpeded activation of the XLas isoforms. Defects in GNAS are a cause of ACTH-independent macronodular adrenal hyperplasia (AIMAH) [MIM:219080]; also known as adrenal Cushing syndrome due to AIMAH. A rare adrenal defect characterized by multiple, bilateral, non-pigmented, benign, adrenocortical nodules. It results in excessive production of cortisol leading to ACTH-independent Cushing syndrome. Clinical manifestations of Cushing syndrome G蛋白αS抗体include facial and trunkal obesity, abdominal striae, muscular weakness, osteoporosis, arterial hypertension, diabetes. Genetic variations in GNAS are the cause of pseudohypoparathyroidism type 1B (PHP1B) [MIM:603233]. PHP1B is characterized by parathyroid hormone (PTH)-resistant hypocalcemia and hyperphosphatemia. Patients affected with PHP1B have normal activity of the product of GNAS, lack developmental defects characteristic of AHO, and typically show no other endocrine abnormalities besides resistance to PTH. Note=Most affected individuals have defects in methylation of the gene. In some cases microdeletions involving the STX16 appear to cause loss of methylation at exon A/B of GNAS, resulting in PHP1B. Paternal uniparental isodisomy have also been observed. Defects in GNAS are the cause of pseudohypoparathyroidism type 1C (PHP1C)G蛋白αS抗体 [MIM:612462]. It is a disorder characterized by end-organ resistance to parathyroid hormone, hypocalcemia and hyperphosphatemia. It is commonly associated with Albright hereditary osteodystrophy whose features are short stature, obesity, round facies, short metacarpals and ectopic calcification. Similarity: Belongs to the G-alpha family. G(s) subfamily. membrane protein. Gene ID: 2778 Database links: Entrez Gene: 281793 Cow Entrez Gene: 2778 Human Entrez Gene: 14683 Mouse Entrez Gene: 100049657 Pig Entrez Gene: 24896 Rat Omim: 139320 Human SwissProt: P04896 Cow SwissProt: P63091 Dog SwissProt: P63092 Human SwissProt: P84996 Human SwissProt: Q5JWF2 Human SwissProt: P63094 Mouse SwissProt: Q6R0H7 Mouse SwissProt: P29797 Pig SwissProt: P63095 Rat SwissProt: Q63803 Rat Unigene: 125898 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
G蛋白αS抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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：肿瘤 细胞生物 **学 转录调节因子 结合蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid