GAA anticorps (AA 174-203)

N° du produit ABIN7871285

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB et IHC (p). Il présente une réactivité avec des échantillons de Humain.

Aperçu rapide pour GAA anticorps (AA 174-203) (ABIN7871285)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Détail du produit anti-GAA anticorps

Épitope: AA 174-203

Fonction: GAA Antibody / Glucosidase alpha acid

Purification: Antigen affinity purified

Immunogène: A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.

Isotype: Ig Fraction

Information d'application

Indications d'application: The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: In 1X PBS, pH 7.4, with 0.09 % sodium azide

Agent conservateur: Sodium azide

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

Stock: -20 °C

Stockage commentaire: Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Détails sur GAA

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

Autre désignation: GAA

Sujet: 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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process