葡萄糖-6-磷酸酶3/G6Pase-β抗体
规格：1mg/1ml
英文名： G6PC3
别名： 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; U
分子量： 39kDa
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 G6PC3
交叉反应：Human, Mouse, Rat, Cow, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜
葡萄糖-6-磷酸酶3/G6Pase-β抗体产品介绍：background: 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 葡萄糖-6-磷酸酶3/G6Pase-β抗体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 葡萄糖-6-磷酸酶3/G6Pase-β抗体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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
葡萄糖-6-磷酸酶3/G6Pase-β抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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：细胞生物 信号转导 糖尿病 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid