GAA Polyclonal Antibody, Biotin Conjugated, Rabbit

Product Overview

• Article Name: GAA Polyclonal Antibody, Biotin Conjugated, Rabbit
• Biozol Catalog Number: BSS-BS-13254R-BIOTIN
• Supplier Catalog Number: bs-13254R-Biotin
• Alternative Catalog Number: BSS-BS-13254R-BIOTIN-100
• Manufacturer: Bioss
• Host: Rabbit
• Category: Antikörper
• Application: ELISA, IHC-Fr, IHC-P, WB
• Species Reactivity: Human, Mouse
• Conjugation: Biotin
• Alternative Names: 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.

Product Description

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 Pompes 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].

Product Properties

• Clonality: Polyclonal
• Concentration: 1ug/ul
• NCBI: 2548
• UniProt: P10253
• Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
• Source: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
• Target: GAA

Application Notes

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