GAA anticorps (AA 541-640) (HRP)

N° du produit ABIN1712134

Détail du produit anti-GAA anticorps (HRP)

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

Épitope: AA 541-640

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

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

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Réactivité croisée: Humain, Souris, Rat

Homologie: Dog,Sheep,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Conseil sur la manipulation: Do NOT add Sodium Azide! Use of Sodium Azide will inhibit enzyme activity of horseradish peroxidase.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

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:

Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.

Background: 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. [provided by RefSeq, Jul 2008].

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process