GAA Polyclonal Antibody, Biotin Conjugated, Rabbit

Produktübersicht

• Artikelname: GAA Polyclonal Antibody, Biotin Conjugated, Rabbit
• Artikelnummer: BSS-BS-13254R-BIOTIN
• Hersteller Artikelnummer: bs-13254R-Biotin
• Alternativnummer: BSS-BS-13254R-BIOTIN-100
• Hersteller: Bioss
• Wirt: Rabbit
• Kategorie: Antikörper
• Applikation: ELISA, IHC-Fr, IHC-P, WB
• Spezies Reaktivität: Human, Mouse
• Konjugation: Biotin
• Alternative Synonym: 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.

Produktbeschreibung

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].

Produkteigenschaften

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

Anwendungsbeschreibung

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