Rabbit Anti-G6PC3 antibody | Sunlong Medical

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G-6-Pase 3; G6Pase 3; G6Pase-beta; Glucose 6 phosphatase, catalytic, 3; Glucose-6-phosphatase 3; Glucose-6-phosphatase beta; SCN4; Ubiquitous glucose-6-phosphatase catalytic subunit-related protein; UGRP;

Cat:

SL13253R

Species Reactivity：

Human,Mouse,Rat,(predicted: Cow,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human G6PC3:201-300/346

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Sample: A549(Human) Cell Lysate at 30 ugHepG2(Human) Cell Lysate at 30 ugPrimary: Anti- G6PC3 (SL13253R) at 1/1000 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 39 kDObserved band size: 39 kDSample: Muscle (Mouse) Lysate at 40 ugPrimary: Anti-G6PC3 (SL13253R) at 1/1000 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 39 kDObserved band size: 39 kDSample: Skeletal muscle (Mouse) Lysate at 40 ugSkeletal muscle (Rat) Lysate at 40 ugPrimary: Anti- G6PC3 (SL13253R) at 1/1000 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 39 kDObserved band size: 39 kD

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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. Multiple transcript variants have been found for this gene, only one of which is expected to express a protein.[provided by RefSeq, Sep 2009].

Function:
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.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitously expressed. Highly expressed in skeletal muscle, at intermediate levels in heart, brain, placenta, kidney, colon, thymus, spleen and pancreas. Also detected in testis, prostate, ovary, liver, lung, small intestine and peripheral blood lymphocytes.

DISEASE:
Defects in G6PC3 are the cause of neutropenia severe congenital autosomal recessive type 4 (SCN4) [MIM:612541]. 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) [MIM:612541]. A disease characterized by pulmonary arterial hypertension, cardiac abnormalities including secundum-type atrial septal defect, intermittent neutropenia, lymphopenia, monocytosis and anemia.

Similarity:
Belongs to the glucose-6-phosphatase family.

SWISS:
Q9BUM1

Gene ID:
92579

Database links:

Entrez Gene: 92579 Human

Entrez Gene: 61681 Mouse

Entrez Gene: 303565 Rat

Omim: 611045 Human

SwissProt: Q9BUM1 Human

SwissProt: Q6NSQ9 Mouse

SwissProt: Q6AZ83 Rat

Picture

Sample:
Muscle (Mouse) Lysate at 40 ug
Primary: Anti-G6PC3 (SL13253R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 39 kD
Observed band size: 39 kD

Sample:
Skeletal muscle (Mouse) Lysate at 40 ug
Skeletal muscle (Rat) Lysate at 40 ug
Primary: Anti- G6PC3 (SL13253R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 39 kD
Observed band size: 39 kD

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