GAA anticorps (AA 494-527)

N° du produit ABIN5647392

Détail du produit anti-GAA anticorps

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

Épitope: AA 494-527

Reactivité: Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Purification: Antigen affinity purified

Immunogène: Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.

Isotype: IgG

Information d'application

Indications d'application: Optimal dilution of the GAA antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,Immunohistochemistry (FFPE): 1-2 μg/mL

Restrictions: For Research Use only

Stockage

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Stock: -20 °C

Stockage commentaire: After reconstitution, the GAA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Détails sur GAA

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

Autre désignation: GAA / Glucosidase alpha acid (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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process