GAA antibody (AA 541-640)

Catalog No. ABIN1714619

The Rabbit Polyclonal anti-GAA antibody is suitable to detect GAA in samples from Human. It has been validated for WB, IF (cc), IF (p), IHC (p) and IHC (fro).

Quick Overview for GAA antibody (AA 541-640) (ABIN1714619)

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Product Details anti-GAA Antibody

Binding Specificity: AA 541-640

Purpose: GAA Polyclonal Antibody

Cross-Reactivity: Human, Mouse

Predicted Reactivity: Rat,Dog,Sheep,Pig,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

Isotype: IgG

Application Details

Application Notes: WB(1:300-5000),

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Alternative Name: GAA

Background:

Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.

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. [provided by RefSeq, Jul 2008].

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process