GPLL; GPBB; Hers Disease; Glycogen Storage Disease Type VI

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

Monoclonal

IgG2a Kappa

13#

Mouse

The antibody is a?Mouse monoclonal?antibody raised against?PYGL. It has been selected for its ability to recognize?PYGL in immunohistochemical staining and western blotting.

Protein A/G Affinity Chromatography.

Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.

500ug/mL (lot specific)

Storage: Avoid repeated freeze/thaw cycles.
Store at 4 degree C for frequent use.
Aliquot and store at -20 degree C for 12 months.
Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-PYGL 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.

WB; IHC; ICC; IP.

Western blotting: 0.5-5μg/mL
Immunocytochemistry in formalin fixed cells: 5-30μg/mL
Immunohistochemistry in formalin fixed frozen section: 5-30μg/mL
Immunohistochemistry in paraffin section: 5-30μg/mL

Optimal working dilutions must be determined by end user

Western Blot: Sample: Recombinant PYGL, Rat.

97,483 Da

glycogen phosphorylase L

glycogen phosphorylase, liver form

Glycogen phosphorylase, liver form

Lgp??[Similar Products]

catalyzes the rate-limiting step of glycogen catabolism [RGD, Feb 2006]

PYGL: Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties. Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6). A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected. Belongs to the glycogen phosphorylase family.

Protein type: Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.1; Transferase

Chromosomal Location of Human Ortholog: 6q24

Cellular Component: cytoplasm

Molecular Function: AMP binding; ATP binding; bile acid binding; carbohydrate binding; drug binding; glycogen phosphorylase activity; protein homodimerization activity; purine binding; pyridoxal phosphate binding; vitamin binding

Biological Process: 5-phosphoribose 1-diphosphate biosynthetic process; glucose homeostasis; glycogen catabolic process; glycogen metabolic process

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