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Rabbit Anti-GNAS  antibody (bs-3939R)  
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產品編號 bs-3939R
英文名稱 Rabbit Anti-GNAS  antibody
中文名稱 GNAS抗體
別    名 GNAS1_HUMAN; Guanine nucleotide-binding protein G(s) subunit alpha isoforms XLas; Adenylate cyclase-stimulating G alpha protein; Extra large alphas protein; XLalpha; GNAS complex locus; AHO; GSA; GSP; POH; GPSA; NESP; SCG6; SgVI; GNAS1; PITA3; C20orf45;   鳥嘌呤核苷酸結合蛋白G(s)亞單位α亞型XLas;
Specific References  (1)     |     bs-3939R has been referenced in 1 publications.
[IF=0.6] Yin, Zhihong, et al. "Identification of differentially expressed Gnαs and Gnα11 in sheep (Ovis aries) skins associated with white and black coat colors." Acta histochemica (2016).  WB ;  Sheep.  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  結合蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse,Rat (predicted: Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse,Danio rerio)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 111kDa
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GNAS: 901-1037/1037 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q5JWF2

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



產品圖片
Sample: Lane 1: Human MCF-7 cell lysates Lane 2: Human HepG2 cell lysates Primary: Anti-GNAS (bs-3939R) at 1/500 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 111 kDa Observed band size: 130 kDa
Tissue/cell: human lung carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-G protein alpha S Polyclonal Antibody, Unconjugated(bs-3939R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-G protein alpha S Polyclonal Antibody, Unconjugated(bs-3939R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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