GAA antibody (C-Term)

Catalog No. ABIN7466569

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

Quick Overview for GAA antibody (C-Term) (ABIN7466569)

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

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

Grade: KO Validated

Product Details anti-GAA Antibody

Binding Specificity: C-Term

Cross-Reactivity: Human

Purification: Purified by antigen-affinity chromatography.

Immunogen: Recombinant protein encompassing a sequence within the C-terminus region of human LYAG. The exact sequence is proprietary.

Isotype: IgG

Application Details

Application Notes: WB: 1:500-1:3000. IHC-P: 1:100-1:1000. Optimal dilutions/concentrations should be determined by the researcher. Not tested in other applications.

Comment:

Validation: KO/KD

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.76 mg/mL

Buffer: 1XPBS pH 7, 20 % Glycerol, 0.025 % ProClin 300

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: Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4°C. For long-term storage, aliquot and store at -20°C or below. Avoid multiple freeze-thaw cycles.

Target Details for GAA

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

Alternative Name: glucosidase alpha, acid

Background: Glucosidase alpha, acid , LYAG,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]

Molecular Weight: 105 kDa

Gene ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process