AGLU Antikörper (N-Term)

Produktnummer ABIN950380

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch AGLU in WB, EIA und IHC (p). Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für AGLU Antikörper (N-Term) (ABIN950380)

Target: AGLU (alpha-Glucosidase (AGLU))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser AGLU Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Enzyme Immunoassay (EIA), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Produktdetails anti-AGLU Antikörper

Bindungsspezifität: AA 173-203, N-Term

Spezifität: This antibody recognizes Human Alpha-glucosidase (N-term).

Aufreinigung: Protein A column, followed by peptide affinity purification

Immunogen: conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of Human Alpha-glucosidase Genename: GAA

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.25 mg/mL

Buffer: PBS containing 0.09 % (W/V) Sodium Azide as preservative

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Details zu AGLU

Target: AGLU (alpha-Glucosidase (AGLU))

Andere Bezeichnung: alpha-Glucosidase

Hintergrund: 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.Synonyms: Acid maltase, Aglucosidase alfa, GAA, LYAG, Lysosomal alpha-glucosidase

Molekulargewicht: 105319 Da

Gen-ID: 2548

NCBI Accession: NP_000143

Pathways: Cellular Glucan Metabolic Process