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G6PC3 Rabbit pAb (bs-13253R)  
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產(chǎn)品編號(hào) bs-13253R
英文名稱 G6PC3 Rabbit pAb
中文名稱 葡萄糖-6-磷酸酶3/G6Pase-β抗體
別    名 G-6-Pase 3; G6Pase 3; G6Pase-beta; Glucose 6 phosphatase, catalytic, 3; Glucose-6-phosphatase 3; Glucose-6-phosphatase beta; SCN4; Ubiquitous glucose-6-phosphatase catalytic subunit-related protein; UGRP;  
Specific References  (3)     |     bs-13253R has been referenced in 3 publications.
[IF=6.117] Jiahui Li. et al. Dietary iron modulates hepatic glucose homeostasis via regulating gluconeogenesis. J NUTR BIOCHEM. 2022 Nov;109:109104  WB ;  Mouse, Rat.  
[IF=4.129] Jiang, Bin. et al. Role of Proximal Intestinal Glucose Sensing and Metabolism in the Blood Glucose Control in Type 2 Diabetic Rats After Duodenal Jejunal Bypass Surgery. Obes Surg. 2022 Jan;:1-11  WB ;  Rat.  
[IF=2.718] Naomi Nishio. et al. Hen egg only diets support healthy aging in adult mice. J ANIM PHYSIOL AN N. 2023 Jan;:  WB ;  Mouse.  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  糖尿病  新陳代謝  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse,Rat (predicted: Rabbit,Sheep,Cow)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 39 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human G6PC3: 201-300/346 
亞    型
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(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 This gene encodes the catalytic subunit of glucose-6-phosphatase (G6Pase). G6Pase is located in the endoplasmic reticulum (ER) and catalyzes the hydrolysis of glucose-6-phosphate to glucose and phosphate in the last step of the gluconeogenic and glycogenolytic pathways. Mutations in this gene result in autosomal recessive severe congenital neutropenia. Multiple transcript variants have been found for this gene, only one of which is expected to express a protein.[provided by RefSeq, Sep 2009].

Function:
Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. May form with the glucose-6-phosphate transporter (SLC37A4/G6PT) a ubiquitously expressed complex responsible for glucose production through glycogenolysis and gluconeogenesis. Probably required for normal neutrophil function.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitously expressed. Highly expressed in skeletal muscle, at intermediate levels in heart, brain, placenta, kidney, colon, thymus, spleen and pancreas. Also detected in testis, prostate, ovary, liver, lung, small intestine and peripheral blood lymphocytes.

DISEASE:
Defects in G6PC3 are the cause of neutropenia severe congenital autosomal recessive type 4 (SCN4) [MIM:612541]. Autosomal recessive SCN constitutes a primary immunodeficiency syndrome associated with increased apoptosis in myeloid cells. Individuals show a paucity of mature neutrophils in peripheral blood and bone marrow and develop life-threatening bacterial infections. SCN4 is a severe congenital neutropenia syndrome associated with cardiac and urogenital malformations.
Defects in G6PC3 are the cause of Dursun syndrome (DURSS) [MIM:612541]. A disease characterized by pulmonary arterial hypertension, cardiac abnormalities including secundum-type atrial septal defect, intermittent neutropenia, lymphopenia, monocytosis and anemia.

Similarity:
Belongs to the glucose-6-phosphatase family.

SWISS:
Q9BUM1

Gene ID:
92579

Database links:

Entrez Gene: 92579 Human

Entrez Gene: 68401 Mouse

Entrez Gene: 303565 Rat

Omim: 611045 Human

SwissProt: Q9BUM1 Human

SwissProt: Q6NSQ9 Mouse

SwissProt: Q6AZ83 Rat



產(chǎn)品圖片
Sample: A549(Human) Cell Lysate at 30 ug HepG2(Human) Cell Lysate at 30 ug Primary: Anti- G6PC3 (bs-13253R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 39 kD Observed band size: 39 kD
Sample: Skeletal muscle (Mouse) Lysate at 40 ug Skeletal muscle (Rat) Lysate at 40 ug Primary: Anti- G6PC3 (bs-13253R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 39 kD Observed band size: 39 kD
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