GAA antibody

Catalog No. ABIN7263675

Product Details anti-GAA Antibody

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)

Characteristics: Polyclonal Antibody

Purification: Affinity purification

Immunogen: A synthetic peptide of human GAA (NP_000143.2).

Isotype: IgG

Application Details

Application Notes: WB 1:500-1:2000

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3

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. Avoid freeze / thaw cycles.

Target Details for GAA

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

Alternative Name: GAA (GAA Products)

Synonyms: LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody

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.

Molecular Weight:

Observed_MW: 105 kDa

Calculated_MW: 105 kDa

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process