G6PC3, Polyclonal Antibody

Glucose-6-phosphatase 3; G-6-Pase 3; G6Pase 3; 3.1.3.9; Glucose-6-phosphatase beta; G6Pase-beta; Ubiquitous glucose-6-phosphatase catalytic subunit-related protein; G6PC3; UGRP; Purified Rabbit Polyclonal Antibody (Pab)

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Ig

Rabbit

This G6PC3 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 215-246 amino acids from human G6PC3.

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

1.49 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-G6PC3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

All lanes : Anti-G6PC3 Antibody (C-Term) at 1:1000 dilution
Lane 1: Hela whole cell lysate
Lane 2: A549 whole cell lysate
Lane 3: mouse brain lysate
Lysates/proteins at 20 ug per lane.
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution.
Predicted band size : 39 kDa
Blocking/Dilution buffer: 5% NFDM/TBST.

38735 Da

glucose-6-phosphatase catalytic subunit 3

glucose-6-phosphatase 3

Glucose-6-phosphatase 3

UGRP; G-6-Pase 3; G6Pase 3; G6Pase-beta  [Similar Products]

G6PC3_HUMAN

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]

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, SLC family; Membrane protein, multi-pass; Phosphatase (non-protein); Membrane protein, integral; EC 3.1.3.9; Transporter

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: gluconeogenesis; glucose 6-phosphate metabolic process; glucose transport

Disease: Neutropenia, Severe Congenital, 4, Autosomal Recessive

Mural R.J.,et al.Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases. Martin C.C.,et al.J. Mol. Endocrinol. 29:205-222(2002). Guionie O.,et al.FEBS Lett. 551:159-164(2003). Shieh J.-J.,et al.J. Biol. Chem. 278:47098-47103(2003). Ghosh A.,et al.J. Biol. Chem. 279:12479-12483(2004).

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