G6PC Antibody, Biotin conjugated

Code CSB-PA009118LD01HU
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Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) G6PC Polyclonal antibody
Uniprot No. P35575
Target Names G6PC
Alternative Names 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
Raised in Rabbit
Species Reactivity Human
Immunogen Recombinant Human Glucose-6-phosphatase protein (82-117AA)
Immunogen Species Homo sapiens (Human)
Conjugate Biotin
Clonality Polyclonal
Isotype IgG
Purification Method >95%, Protein G purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form Liquid
Tested Applications ELISA
Protocols ELISA Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time 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.

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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease Glycogen storage disease 1A (GSD1A)
Subcellular Location Endoplasmic reticulum membrane; Multi-pass membrane protein.
Protein Families Glucose-6-phosphatase family
Database Links

HGNC: 4056

OMIM: 232200

KEGG: hsa:2538

STRING: 9606.ENSP00000253801

UniGene: Hs.212293

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