| 產(chǎn)品編號 | bs-3939R-AP |
| 英文名稱 | Rabbit Anti-G protein alpha S/NESP55/AP Conjugated antibody |
| 中文名稱 | 堿性磷酸酶（AP）標(biāo)記的G蛋白αS抗體（鳥嘌呤核苷酸結(jié)合蛋白Gα s） |
| 別 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 nucleotide binding protein (G protein) alpha stimulating activity polypeptide 1; Guanine nucleotide binding protein alpha stimulating activity polypeptide 1; Guanine nucleotide binding protein G(s) subunit alpha isoforms short; Guanine nucleotide binding protein G(s) subunit alpha isoforms XLas; Guanine nucleotide regulatory protein; MGC33735; NESP; NESP55; Neuroendocrine secretory protein; PHP1A; PHP1B; POH; Protein ALEX; SCG6; Secretogranin VI; XLalphas; XLas; C20orf45; dJ309F20.1.1; dJ806M20.3.3; GNAS1_HUMAN. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 轉(zhuǎn)錄調(diào)節(jié)因子 結(jié)合蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, Danio rerio) |
| 產(chǎn)品應(yīng)用 | WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 111kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human G protein alpha S |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 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: 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 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) [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. Database links: Entrez Gene: 2778 Human Entrez Gene: 14683 Mouse Omim: 139320 Human SwissProt: P63092 Human SwissProt: P84996 Human SwissProt: Q5JWF2 Human SwissProt: P63094 Mouse SwissProt: Q6R0H7 Mouse Unigene: 125898 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
