GAA anticorps (Middle Region)

N° du produit ABIN4886596

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB et IHC. Il présente une réactivité avec des échantillons de Humain.

Aperçu rapide pour GAA anticorps (Middle Region) (ABIN4886596)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Détail du produit anti-GAA anticorps

Épitope: Middle Region

Fonction: Anti-GAA Antibody

Séquence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

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.

Attributs du produit: Anti-GAA Antibody. Tested in IHC, WB applications. This antibody reacts with Human.

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: Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human Western blot, 0.1-0.5 μg/mL, Human

Commentaires:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg 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.

Conseil sur la manipulation: Avoid repeated freezing and thawing.

Stock: 4 °C,-20 °C

Stockage commentaire: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

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

Poids moléculaire: 110 kDa, 95kDa, 76kDa, 70 kDa

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process