GAA antibody (AA 432-482)

Catalog No. ABIN8121679

The Rabbit Polyclonal anti-GAA antibody (ABIN8121679) specifically detects GAA in WB. The antibody is reactive with Human, Mouse and Rat samples.

Quick Overview for GAA antibody (AA 432-482) (ABIN8121679)

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

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-GAA Antibody

Binding Specificity: AA 432-482

Purpose: LYAG Antibody

Specificity: This antibody detects endogenous levels of LYAG at Human/Mouse/Rat

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Immunogen: Synthesized peptide derived from human LYAG AA range: 432-482

Isotype: IgG

Application Details

Application Notes: WB 1:500-2000

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Liquid in PBS containing 50 % glycerol, 0.5 % BSA and 0.02 % sodium azide.

Preservative: Sodium azide

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

Storage: -20 °C

Storage Comment: Store at -20°C/1 year

Expiry Date: 12 months

Target Details for GAA

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

Alternative Name: LYAG

Background:

Synonyms: Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase]

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],

Gene Name: GAA

Protein Name: LYAG

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process