GAA anticorps (AA 432-482)

N° du produit ABIN8121679

Cet anticorps anti-GAA Polyclonal Lapin (ABIN8121679) détecte spécifiquement GAA dans WB. L’anticorps est réactif avec des échantillons de Humain, Souris et Rat.

Aperçu rapide pour GAA anticorps (AA 432-482) (ABIN8121679)

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-GAA anticorps

Épitope: AA 432-482

Fonction: LYAG Antibody

Specificité: This antibody detects endogenous levels of LYAG at Human/Mouse/Rat

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Immunogène: Synthesized peptide derived from human LYAG AA range: 432-482

Isotype: IgG

Information d'application

Indications d'application: WB 1:500-2000

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Liquid in PBS containing 50 % glycerol, 0.5 % BSA and 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: Store at -20°C/1 year

Date de péremption: 12 months

Détails sur GAA

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

Autre désignation: LYAG

Sujet:

Synonyms: Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase]

Background: 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. [provided by RefSeq, Jan 2016],

Gene Name: GAA

Protein Name: LYAG

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process