GAA anticorps

N° du produit ABIN7263675

Détail du produit anti-GAA anticorps

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)

Attributs du produit: Polyclonal Antibody

Purification: Affinity purification

Immunogène: A synthetic peptide of human GAA (NP_000143.2).

Isotype: IgG

Information d'application

Indications d'application: WB 1:500-1:2000

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

Buffer: PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3

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. Avoid freeze / thaw cycles.

Détails sur GAA

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

Autre désignation: GAA (GAA Produits)

Synonymes: anticorps LYAG, anticorps E430018M07Rik, anticorps glucosidase alpha, acid, anticorps glucosidase, alpha, acid, anticorps transmembrane and coiled-coil domain family 1, anticorps glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II), anticorps GAA, anticorps Gaa, anticorps TMCC1, anticorps gaa

Sujet: 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.

Poids moléculaire:

Observed_MW: 105 kDa

Calculated_MW: 105 kDa

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process