GAA Recombinant Rabbit Monoclonal Antibody [JE54-59]

About this product

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.

Specifications

Catalog number

ET7110-77

Size / count

100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9000-83-3

Product Name Alternative

70 kDa lysosomal alpha-glucosidase antibody Acid alpha glucosidase antibody Acid maltase antibody Aglucosidase alfa antibody Alpha glucosidase antibody GAA antibody Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody Glucosidase alpha acid antibody Glucosidase alpha antibody LYAG antibody LYAG_HUMAN antibody Lysosomal alpha glucosidase antibody

Abbreviation

GAA

Swiss Prot

P10253 Human

Cellular Locus

Lysosome, lysosome membrane.

Host

Rabbit

Species Reactivity

Human

Immunogen

Recombinant protein within Human GAA aa 120-230 / 952.

Isotype

IgG

Conjugation

Non-conjugated

Type

Recombinant Rabbit monoclonal Antibody

Applications

WB, IF-Tissue, IHC-P

Positive Control

HepG2 cell lysate, MCF-7 cell lysate, human placenta tissue lysate, human liver tissue, human placenta tissue, human liver tissue, human liver carcinoma tissue, HepG2.

Concentration

1 mg/mL

Dilution

WB:1:500-1:2,000 ;IF-Tissue: 1:50-1:200 ;IHC-P:1:50-1:1,000

Purity

Protein A affinity purified.

Form

Liquid

Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Molecular Weight

Predicted band size: 105/76/70 kDa.

Storage Conditions

Store at +4°C after thawing. Aliquot store at -20°C. Avoid repeated freeze/thaw cycles.

Recombinant Antibody

Yes