GAA anticorps

N° du produit ABIN7880880

L’anticorps anti-GAA Monoclonal Souris est utilisé pour la détection de GAA dans des échantillons de Humain. Il a été validé pour WB, IHC (p) et IF.

Aperçu rapide pour GAA anticorps (ABIN7880880)

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

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Clone: 2G7

Détail du produit anti-GAA anticorps

Fonction: Glucosidase Alpha Acid Antibody / GAA

Séquence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

Purification: Affinity purified

Immunogène: Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.

Isotype: IgG2b

Information d'application

Indications d'application: Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Stock: 4 °C,-20 °C

Stockage commentaire: After reconstitution, the Glucosidase Alpha Acid antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Détails sur GAA

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

Autre désignation: Glucosidase Alpha Acid

Sujet: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process