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GAA Recombinant Rabbit mAb

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. [provided by RefSeq, Jan 2016]

CAS Number

9000-83-3

Structure Composition

Store at -20°C. Supplied in 50mM Tris-Glycine (pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.

Product Name Alternative

LYAG

Swiss Prot

P10253

Reactivity

Human, Mouse, Rat

Immunogen

A synthetic peptide of human GAA

Conjugation

Unconjugated

Applications

WB

Dilution

WB: 1:1000-1:5000

Purity

Affinity Purification

Modification

Unmodification

Molecular Weight

76 kDa

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

IgG

Applications Notes

Western blot analysis of extracts from 293T cells and Human placenta tissue using BS45947 at 1: 1000.

Host or Source

Rabbit

Available Sizes

Frequently Asked Questions

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