GAA anticorps (N-Term)

N° du produit ABIN2797807

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB et IHC. Il présente une réactivité envers Humain.

Aperçu rapide pour GAA anticorps (N-Term) (ABIN2797807)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Détail du produit anti-GAA anticorps

Épitope: AA 174-203, N-Term

Fonction: Rabbit Anti-Human GAA (N-term) Antibody

Immunogène: This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Isotype: Ig Fraction

Information d'application

Indications d'application: Western Blot, Immunohistochemistry
Recommended Dilutions
WB: 1:1000, IHC: 1:10-50GAA Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.460 mg/mL

Stock: 4 °C,-20 °C

Stockage commentaire: 2-8°C (short-term), -20°C (long-term)

Détails sur GAA

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

Autre désignation: GAA

Sujet:

Target 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 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.

Gene Symbol: GAA

Poids moléculaire: 105324 Da

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process