GAA Antibody

Product Specifications

Background

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

NCBI Gene ID

2548

Swiss Prot

P10253

Accession Number

P10253

Host

Rabbit

Reactivity

Human

Immunogen

This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Obesity, Signal Transduction

Purification

This antibody is purified through a protein A column, followed by peptide affinity purification.

Concentration

Batch dependent

Buffer

Supplied in PBS with 0.09% (W/V) sodium azide.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Calculated Molecular Weight

105 kDa

Fragment

Rabbit Ig

Symbol

GAA

NCBI Official Name

Lysosomal alpha-glucosidase

NCBI Organism

Homo sapiens

Background Reference 01

Bailey, S.D., et al. Diabetes Care 33 (10) :2250-2253 (2010)

Background Reference 02

Labrousse, P., et al. Mol. Genet. Metab. 99 (4) :379-383 (2010)

Background Reference 03

Talmud, P.J., et al. Am. J. Hum. Genet. 85 (5) :628-642 (2009)

Background Reference 04

Aoyama, Y., et al. J. Hum. Genet. 54 (11) :681-686 (2009)

Other Product Names

Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA

Tested Applications

WB, IHC-P, IHC

Protein ID

317373572

Physical Properties

Liquid

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