GAA anticorps (N-Term)

N° du produit ABIN5533211

Détail du produit anti-GAA anticorps

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

Épitope: AA 174-203, N-Term

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Purification: This antibody is purified through a protein A column, followed by peptide affinity purification.

Immunogène: This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

Isotype: Ig Fraction

Information d'application

Indications d'application: For IHC starting dilution is: 1:25

For WB starting dilution is: 1:1000

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: Supplied in PBS with 0.09 % (W/V) 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.

Stock: 4 °C,-20 °C

Stockage commentaire: Store at 4°C for three months and -20°C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

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: 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.

Poids moléculaire: 105 kDa

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process