Rabbit anti-Human G6PC3 Polyclonal Antibody | anti-G6PC3 antibody
G6PC3 Rabbit Polyclonal
Immunogen Affinity Purified
Immunogen Affinity Purified
NCBI and Uniprot Product Information
NCBI Description
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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2016]
Uniprot Description
G6PC3: 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. Defects in G6PC3 are the cause of neutropenia severe congenital autosomal recessive type 4 (SCN4). 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). A disease characterized by pulmonary arterial hypertension, cardiac abnormalities including secundum-type atrial septal defect, intermittent neutropenia, lymphopenia, monocytosis and anemia. Belongs to the glucose-6-phosphatase family.
Protein type: Transporter; Membrane protein, integral; EC 3.1.3.9; Phosphatase (non-protein); Transporter, SLC family; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 17q21.31
Cellular Component: endoplasmic reticulum membrane; integral to endoplasmic reticulum membrane; membrane
Molecular Function: glucose-6-phosphatase activity
Biological Process: dephosphorylation; gluconeogenesis; glucose 6-phosphate metabolic process; glucose transport; glucose-6-phosphate transport
Disease: Neutropenia, Severe Congenital, 4, Autosomal Recessive
Research Articles on G6PC3
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Product Notes
The G6PC3 g6pc3 (Catalog #AAA769500) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The G6PC3 Rabbit Polyclonal reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's G6PC3 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC). Researchers should empirically determine the suitability of the G6PC3 g6pc3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "G6PC3, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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