Anti-GAA Rabbit Polyclonal Antibody
Catalog # BOSSBS-13254R
Supplier: Bioss
Specifications
- Antibody type:Primary
- Antigen name:Glucosidase Acid, Alpha
- Antigen symbol:GAA
- Clonality:Polyclonal
- Conjugation:Unconjugated
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western blot:Yes
- Environmentally Preferable:
- Form:liquid
- Gene ID:2548
- Antigen synonyms:LYAG
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0,09% sodium azide. Store at -20°C for 12 months.
- Concentration:1 ug/ul
- Shipping temperature:4 °C
- Purification:Purified by Protein A.
- Size:100 μg
- Pk:100 µl
Specifications
About this item
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, Jul 2008].
WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
Type: Primary
Antigen: GAA
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat