GAA antibody (N-Term)

Catalog No. ABIN5533211

Product Details anti-GAA Antibody

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

Binding Specificity: AA 174-203, N-Term

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

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

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

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.

Isotype: Ig Fraction

Application Details

Application Notes: For IHC starting dilution is: 1:25

For WB starting dilution is: 1:1000

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: Supplied in PBS with 0.09 % (W/V) 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: 4 °C,-20 °C

Storage Comment: 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.

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 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.

Molecular Weight: 105 kDa

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process