GAA anticorps (N-Term)

N° du produit ABIN635720

Détail du produit anti-GAA anticorps

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

Épitope: N-Term

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB)

Specificité: GAA antibody was raised against the N terminal of GAA

Purification: Affinity purified

Immunogène: GAA antibody was raised using the N terminal of GAA corresponding to a region with amino acids FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Information d'application

Indications d'application: WB: 1 µg/mL
Optimal conditions should be determined by the investigator.

Commentaires:

GAA Blocking Peptide, catalog no. 33R-2917, is also available for use as a blocking control in assays to test for specificity of this GAA antibody

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Lyophilized powder. Add distilled water for a 1 mg/mL concentration of GAA antibody in PBS

Concentration: Lot specific

Buffer: PBS

Conseil sur la manipulation: Avoid repeated freeze/thaw cycles.
Dilute only prior to immediate use.

Stock: 4 °C/-20 °C

Stockage commentaire: Store at 2-8 °C for short periods. For longer periods of storage, store at -20 °C.

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: GAA is 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.

Poids moléculaire: 98 kDa (MW of target protein)

Pathways: Cellular Glucan Metabolic Process