Metabolism Antibodies 3

Anti-G6PC3 Antibody (CAB16234)

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SKU:
CAB16234
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-G6PC3 Antibody
Antibody SKU:CAB16234
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human G6PC3 (NP_612396.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:K-562, HeLa, 293T, Mouse kidney, Mouse heart
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human G6PC3 (NP_612396.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:SRRV GIAV LWIS LITE WLNL IFKW FLFG DRPF WWVH ESGY YSQA PAQV HQFP SSCE TGPG SPSG HCMI TGAA LWPI MTAL SSQV ATRA RSRW VRVM PSLA Y
Gene ID:92579
Uniprot:Q9BUM1
Cellular Location:Endoplasmic reticulum membrane, Multi-pass membrane protein
Calculated MW:38kDa
Observed MW:37kDa
Synonyms:G6PC3, SCN4, UGRP
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. Alternative splicing results in multiple transcript variants.
UniProt Protein Function: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.
UniProt Protein Details:

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

NCBI Summary: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 Code:Q9BUM1
NCBI GenInfo Identifier:74733234
NCBI Gene ID:92579
NCBI Accession:Q9BUM1.2
UniProt Secondary Accession:Q9BUM1,Q8WU15,
UniProt Related Accession:Q9BUM1
Molecular Weight:38735
NCBI Full Name:Glucose-6-phosphatase 3
NCBI Synonym Full Names:glucose-6-phosphatase catalytic subunit 3
NCBI Official Symbol:G6PC3  
NCBI Official Synonym Symbols:SCN4; UGRP  
NCBI Protein Information:glucose-6-phosphatase 3
UniProt Protein Name:Glucose-6-phosphatase 3
UniProt Synonym Protein Names:Glucose-6-phosphatase beta; G6Pase-beta; Ubiquitous glucose-6-phosphatase catalytic subunit-related protein
Protein Family:Glucose-6-phosphatase
UniProt Gene Name:G6PC3  
UniProt Entry Name:G6PC3_HUMAN
Anti-G6PC3 Antibody (CAB16234)Western blot analysis of extracts of various cell lines, using G6PC3 antibody (CAB16234) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5min.
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