GAA Polyklonale Antilichaam, Biotinevervoeging

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].

70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid Pompe disease glycogen storage disease type II; Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase.

2548

P10253

Cytoplasm, Cell membrane

Human, Mouse

541-640/952

GAA

Biotin

KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

WB, ELISA, IHC-P, IHC-F

Purified by Protein A.

1µg/µl

WB (1:300-5000), ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500)

Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Unmodified

Store at -20°C for 12 months.

2548

Rat, Dog, Sheep, Pig, Horse