GAA anticorps (Middle Region)

N° du produit ABIN4886596

Détail du produit anti-GAA anticorps

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

Épitope: AA 494-527, Middle Region

Reactivité: Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Fonction: Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.

Séquence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

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

Attributs du produit: Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.
Gene Name: glucosidase alpha, acid
Protein Name: Lysosomal alpha-glucosidase

Purification: Immunogen affinity purified.

Immunogène: A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR), 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: WB: Concentration: 0.1-0.5 μg/mL, Tested Species: Human, Rat
IHC-P: Concentration: 0.5-1 μg/mL, Tested Species: Human, Epitope Retrieval by Heat: Boiling the paraffin sections in 10 mM citrate buffer, pH 6.0, for 20 mins is required for the staining of formalin/paraffin sections.
Notes: Tested Species: Species with positive results. Other applications have not been tested. Optimal dilutions should be determined by end users.

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: At -20°C for one year. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20 °C for a longer time. Avoid repeated freezing and thawing.

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: 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.

Synonyms: Acid alpha glucosidase | Acid maltase | Aglucosidase alfa | Alpha glucosidase | GAA | Glucosidase alpha acid | Glucosidase alpha | LYAG | P10253

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process