GAA antibody

Catalog No. ABIN6989688

This anti-GAA antibody is a Rabbit Monoclonal antibody detecting GAA in WB, IHC (p) and FACS. Suitable for Human.

Quick Overview for GAA antibody (ABIN6989688)

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

Reactivity: Human

Host: Rabbit

Clonality: Monoclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Flow Cytometry (FACS)

Clone: 5H49

Product Details anti-GAA Antibody

Cross-Reactivity: Human

Purification: Purified by Protein A.

Immunogen: Synthetic peptide within Human GAA.

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 1xTBS ( pH 7.4), 1 % BSA, 40 %Glycerol and 0.05 % Sodium Azide.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C for 12 months.

Expiry Date: 12 months

Target Details for GAA

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

Alternative Name: GAA

Background:

Synonyms: 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

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