GAA anticorps (AA 541-640) (HRP)

N° du produit ABIN1712134

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB, ELISA, IHC (p) et IHC (fro). Il présente une réactivité envers Humain, Souris et Rat.

Aperçu rapide pour GAA anticorps (AA 541-640) (HRP) (ABIN1712134)

Antigène: GAA (Glucosidase, Alpha, Acid (GAA))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est conjugé à/à la HRP

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Détail du produit anti-GAA anticorps (HRP)

Épitope: AA 541-640

Réactivité croisée: Humain, Souris, Rat

Homologie: Dog,Sheep,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

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

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Conseil sur la manipulation: Do NOT add Sodium Azide! Use of Sodium Azide will inhibit enzyme activity of horseradish peroxidase.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur GAA

Antigène: GAA (Glucosidase, Alpha, Acid (GAA))

Autre désignation: GAA

Sujet:

Synonyms: 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.

Background: 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].

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process