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G6PC Antibody

  • 货号:
    CSB-PA009118LA01HU
  • 规格:
    ¥440
  • 促销:
    小规格抗体限时一口价
  • 图片:
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA009118LA01HU at dilution of 1:100
  • 其他:

产品详情

  • 产品名称:
    Rabbit anti-Homo sapiens (Human) G6PC Polyclonal antibody
  • Uniprot No.:
    P35575
  • 基因名:
  • 别名:
    AW107337 antibody; G-6-Pase antibody; G6Pase antibody; G6Pase-alpha antibody; g6pc antibody; G6PC_HUMAN antibody; G6PT antibody; Glucose-6-phosphatase alpha antibody; Glucose-6-phosphatase antibody; glucose-6-phosphatase; catalytic subunit antibody; GSD1 antibody; GSD1a antibody; MGC163350 antibody; MGC93613 antibody; RP23-281C18.19 antibody
  • 宿主:
    Rabbit
  • 反应种属:
    Human
  • 免疫原:
    Recombinant Human Glucose-6-phosphatase protein (82-117AA)
  • 免疫原种属:
    Homo sapiens (Human)
  • 标记方式:
    Non-conjugated

    本页面中的产品,G6PC Antibody (CSB-PA009118LA01HU),的标记方式是Non-conjugated。对于G6PC Antibody,我们还提供其他标记。见下表:

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA009118LB01HU G6PC Antibody, HRP conjugated ELISA
    FITC CSB-PA009118LC01HU G6PC Antibody, FITC conjugated
    Biotin CSB-PA009118LD01HU G6PC Antibody, Biotin conjugated ELISA
  • 克隆类型:
    Polyclonal
  • 抗体亚型:
    IgG
  • 纯化方式:
    >95%, Protein G purified
  • 浓度:
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式:
    Liquid
  • 应用范围:
    ELISA, IHC
  • 推荐稀释比:
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols:
  • 储存条件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能:
    Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production in the terminal step of glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels.
  • 基因功能参考文献:
    1. Microarrays revealed that G6PC mRNA was upregulated following GDNF-mediated dopaminergic differentiation of SH-SY5Y cells. Array association analysis showed three downregulated microRNAs that could possibly influence G6PC translation. Although qRT-PCR results were not significant, they did support the microarray findings with regard to trend. Western blotting also confirmed increased G6PC protein expression following GDNF PMID: 28829278
    2. 3'-UTR SNP rs2229611 in G6PC1 affects mRNA stability, expression and Glycogen Storage Disease type-Ia risk PMID: 28502559
    3. crystal structures of the FoxO1 DNA binding domain in complex with the G6PC1 promoter PMID: 28223045
    4. Notch1 expression is reduced and glucose-6-phosphatase and perilipin-5 (G6PC/PLIN5) are upregulated in liver biopsies from nonalcoholic steatohepatitis (NASH) and nonalcoholic fatty liver disease (NAFLD) patients. PMID: 27428080
    5. Mutation analysis of the G6PC gene revealed that GSD Ia accounted for 11% in GSD patients with involvement of liver. Three patients were homozygous for R83C mutation. In addition, a novel stop mutation, Y85X, was identified in a patient with the typical features of GSD Ia. PMID: 28360385
    6. Post-translational regulation of the glucose-6-phosphatase complex by cyclic AMP is a crucial determinant of endogenous glucose production and is controlled by the glucose-6-phosphate transporter. PMID: 26958868
    7. ApoA-IV colocalizes with NR4A1, which suppresses G6Pase and PEPCK gene expression at the transcriptional level, reducing hepatic glucose output and lowering blood glucose. PMID: 26556724
    8. By direct DNA sequencing, three novel G6PC variations were identified which expanded the G6PC mutation spectrum, and provided conclusive genetic evidences for the definitive diagnosis of the Chinese patients. PMID: 24980439
    9. This study is the first to demonstrate a functional relationship between the critical gluconeogenic and glycogenolytic enzyme G6PC with the metabolic adaptations during glioblastoma invasion. PMID: 25001192
    10. The spectrum of mutations in the G6PC gene. PMID: 24355556
    11. Lipopolysaccharide and monophosphoryl lipid A also up-regulated G6PC and PCK1 transcript abundance in a TLR4-dependent manner. PMID: 23465595
    12. Both GSD-1a and G6PT strongly colocalised in perinuclear membranes. showed that GSD1 mutations did neither alter the G6PC or G6PT chimera localisation, nor the interaction between G6PT termini. PMID: 21983240
    13. results reveal a novel link between glucose metabolism and the DNA damage signaling pathway and suggest a possible role for PEPCK and G6P in the DNA damage response PMID: 21733854
    14. data mitigate against G6PD deficiency contributing to stroke risk in individuals with sickle cell anemia. PMID: 21328436
    15. description of G6PC mutations in Thailand patients with glycogen storage disease type Ia PMID: 19832742
    16. we report the results of structure and function studies of the 48 missense mutations and the DeltaF327 codon deletion mutation, grouped as active site, helical, and nonhelical mutations PMID: 11739393
    17. active site of G6Pase: role of HIS176 as the nucleophile forming the phosphohistidine-enzyme intermediate during catalysis PMID: 12093795
    18. homozygosity for one G6PC mutation, G188R, seems to be associated with a glycogen storage disease type I non-a phenotype and homozygosity for the 727G>T mutation may be associated with a milder phenotype but an increased risk for hepatocellular carcinoma PMID: 12373566
    19. The amino-terminal domain of G6PT is required for optimal glucose-6-phosphate uptake activity. PMID: 12444104
    20. maximum repression of basal glucose-6-phosphatase catalytic subunit (G6Pase) gene transcription by insulin requires two distinct promoter regions, designated that together form an insulin response unit. PMID: 12556524
    21. Five mutants lack microsomal G6P uptake activity and one retains residual activity, suggesting that in G6PT the signature motif is a functional element required for microsomal glucose-6-phosphate transport. PMID: 12560945
    22. a novel, widely expressed G6Pase-related protein, PAP2.8/UGRP, renamed here G6Pase-beta couples with the G6P transporter to form an active G6Pase complex that can hydrolyze G6P to glucose PMID: 13129915
    23. Glc-6-Pase-alpha and Glc-6-Pase-beta share a similar active site structure, topology, and mechanism of action PMID: 14718531
    24. G6pc expression was functionally silenced by adenovirus-mediated delivery of short hairpin RNA. PMID: 14759518
    25. Findings suggest that the screening for 727G-->T and R83H mutations of glucose-6-phosphatase gene in conjunction with the 1176 polymorphism linkage analysis is a good method for gene and prenatal diagnosis of glycogen storage disease Ia. PMID: 15696478
    26. HNF4alpha, CREM, HNF1alpha, and C/EBPalpha have roles in transcriptional regulation of the glucose-6-phosphatase gene by cAMP/vasoactive intestinal peptide in the intestine PMID: 16893891
    27. G6PC1 hepatic activity was abnormally low in 98 SIDS (preterm, n=13; term, n=85), and non-SIDS preterm infants (n=35) compared to term non-SIDS infants (n=29) and adults (n=9) PMID: 17354259
    28. analysis of mutation spectrum of glycogen storage disease type Ia in Tunisia PMID: 18008183
    29. summary of the reported G6PC mutations and review what mutagenesis studies have revealed about the structure and function of the G6PC catalytic unit [review] PMID: 18449899
    30. EGF also inhibits hepatic G6Pase gene expression in vivo PMID: 18847435
    31. Identification of a risk conferring single nucleotide polymorphism in G6PC for type 2 diabetes in a Chinese population. PMID: 19082990
    32. Increased transcriptional expression of PEPCK1 and G6Pc does not account for increased gluconeogenesis and fasting hyperglycemia in patients with type 2 diabetes mellitus. PMID: 19587243

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  • 相关疾病:
    Glycogen storage disease 1A (GSD1A)
  • 亚细胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族:
    Glucose-6-phosphatase family
  • 数据库链接:

    HGNC: 4056

    OMIM: 232200

    KEGG: hsa:2538

    STRING: 9606.ENSP00000253801

    UniGene: Hs.212293