GAA anticorps (AA 350-450)

N° du produit ABIN6140865

Détail du produit anti-GAA anticorps

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

Épitope: AA 350-450

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB), Immunoprecipitation (IP), Immunofluorescence (IF)

Séquence: VVQQYLDVVG YPFMPPYWGL GFHLCRWGYS STAITRQVVE NMTRAHFPLD VQWNDLDYMD SRRDFTFNKD GFRDFPAMVQ ELHQGGRRYM MIVDPAISSS G

Réactivité croisée: Humain, Souris, Rat

Attributs du produit: Polyclonal Antibodies

Purification: Affinity purification

Immunogène: A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).

Isotype: IgG

Information d'application

Indications d'application: WB,1:500 - 1:2000,IF,1:50 - 1:200,IP,1:50 - 1:200

Commentaires:

HIGH QUALITY

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.

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: -20 °C

Stockage commentaire: Store at -20°C. Avoid freeze / thaw cycles.

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 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.,GAA,LYAG,Cancer,Signal Transduction,Endocrine & Metabolism,GAA

Poids moléculaire: 105 kDa

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process