Rabbit Anti-Glucose 6 phosphatase alpha/FITC Conjugated antibody

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glucose-6-phosphatase, catalytic subunit; GSD1; AW107337; G-6-Pase; G6Pase; G6Pase-alpha; g6pc; G6PC_HUMAN; G6PT; Glucose-6-phosphatase alpha; Glucose-6-phosphatase; GSD1a; MGC163350; MGC93613; RP23-281C18.19.

Cat:

SL4044R-FITC

Species Reactivity：

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

Immunogen：

KLH conjugated synthetic peptide derived from human Glucose 6 phosphatase alpha

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

39kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Glucose-6-phosphatase (G6Pase) is a multi-subunit integral membrane protein of the endoplasmic reticulum that is composed of a catalytic subunit and transporters for G6P, inorganic phosphate, and glucose. This gene (G6PC) is one of the three glucose-6-phosphatase catalytic-subunit-encoding genes in human: G6PC, G6PC2 and G6PC3. Glucose-6-phosphatase catalyzes the hydrolysis of D-glucose 6-phosphate to D-glucose and orthophosphate and is a key enzyme in glucose homeostasis, functioning in gluconeogenesis and glycogenolysis. Mutations in this gene cause glycogen storage disease type I (GSD1). This disease, also known as von Gierke disease, is a metabolic disorder characterized by severe hypoglycemia associated with the accumulation of glycogen and fat in the liver and kidneys.[provided by RefSeq, Feb 2011]

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 through glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels.

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

DISEASE:
Defects in G6PC are the cause of glycogen storage disease type 1A (GSD1A) [MIM:23440]. A metabolic disorder characterized by impairment of terminal steps of glycogenolysis and gluconeogenesis. Patients manifest a wide range of clinical symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia.

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

Database links:

Entrez Gene: 403492 Dog

Entrez Gene: 2538 Human

Entrez Gene: 14377 Mouse

Entrez Gene: 25634 Rat

SwissProt: O19133 Dog

SwissProt: P35575 Human

SwissProt: P35576 Mouse

SwissProt: P43428 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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