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GAA polyclonal antibody

Product Specifications

Background

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

CAS Number

9007-83-4

Structure Composition

1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Product Name Alternative

GAA; LYAG

Swiss Prot

P10253

Reactivity

Human, Mouse, Rat

Immunogen

A synthetic peptide of human GAA (NP_000143.2) .

Conjugation

Unconjugated

Applications

WB, IF/ICC, IP

Dilution

WB,1:500 - 1:2000|IF/ICC,1:50 - 1:200|IP,1:50 - 1:200

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Modification

Unmodification

Molecular Weight

105KDa

Storage Conditions

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Notes

For research use only, not for use in diagnostic procedure.

Specificity

Polyclonal Antibodies

Host or Source

Rabbit

Available Sizes

Curated Selection

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