GAA Antibody | 23-097

GAA Antibody | 23-097 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human, Mouse, Rat

Homology: N/A

Immunogen: A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2) .

Research Area: Cancer, Signal Transduction

Tested Application: WB, IF, IP

Application: WB: 1:500 - 1:2000
IF: 1:50 - 1:200
IP: 1:50 - 1:200

Specificiy: N/A

Positive Control 1: LO2

Positive Control 2: HT-29

Positive Control 3: 293T

Positive Control 4: A-549

Positive Control 5: DU145

Positive Control 6: Mouse liver

Molecular Weight: Observed: 105kDa

Validation: N/A

Isoform: N/A

Purification: Affinity purification

Clonality: Polyclonal

Clone: N/A

Isotype: IgG

Conjugate: Unconjugated

Physical State: Liquid

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Concentration: N/A

Storage Condition: Store at -20˚C. Avoid freeze / thaw cycles.

Alternate Name: Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA

User Note: Optimal dilutions for each application to be determined by the researcher.

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.