GAA anticorps (Middle Region) (Fluoro488)

N° du produit ABIN7993501

L’anticorps anti-GAA Polyclonal Lapin est utilisé pour la détection de GAA dans des échantillons de Humain. Il a été validé pour FACS.

Aperçu rapide pour GAA anticorps (Middle Region) (Fluoro488) (ABIN7993501)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est conjugé à/à la Fluoro488

Application: Flow Cytometry (FACS)

Détail du produit anti-GAA anticorps (Fluoro488)

Épitope: Middle Region

Fonction: Anti-GAA Antibody Fluoro488 Conjugated

Specificité: No cross reactivity with other proteins.

Réactivité croisée (Details): No cross-reactivity with other proteins

Homologie: different from the related mouse sequence by eight amino acids,and from the related rat sequence by six amino acids.

Purification: Immunogen affinity purified.

Immunogène: A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Isotype: IgG

Information d'application

Indications d'application: Flow Cytometry, Optimal dilutions should be determined by end users.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % 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: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Date de péremption: 12 months

Détails sur GAA

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

Autre désignation: GAA

Sujet:

Background: 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.

Gene Full Name: alpha glucosidase

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process