GAA anticorps (N-Term)

N° du produit ABIN2781771

Cet anticorps anti-GAA Polyclonal Lapin (ABIN2781771) détecte spécifiquement GAA dans WB et IHC. L’anticorps est réactif avec des échantillons de Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Poisson zèbre (Danio rerio), Cheval et Lapin.

Aperçu rapide pour GAA anticorps (N-Term) (ABIN2781771)

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

Reactivité: Humain, Souris, Rat, Boeuf (Vache), Chien, Cobaye, Poisson zèbre (Danio rerio), Cheval, Lapin

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Détail du produit anti-GAA anticorps

Épitope: N-Term

Fonction: GAA Antibody - N-terminal region

Séquence: FGVIVRRQLD GRVLLNTTVA PLFFADQFLQ LSTSLPSQYI TGLAEHLSPL

Homologie: Cow: 100%, Dog: 100%, Guinea Pig: 100%, Horse: 93%, Human: 100%, Mouse: 100%, Rabbit: 79%, Rat: 100%, Zebrafish: 83%

Attributs du produit: This is a rabbit polyclonal antibody against GAA. It was validated on Western Blot using a cell lysate as a positive control.

Purification: Affinity Purified

Immunogène: The immunogen is a synthetic peptide directed towards the N terminal region of human GAA

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Commentaires:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP44226-100UG

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

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: prevent freeze-thaw cycles

Stock: 4 °C,-20 °C

Stockage commentaire: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Détails sur GAA

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

Autre désignation: GAA

Sujet:

Background Information: 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. 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.

Gene Name: Glucosidase, alpha, acid

Alternative Symbols: LYAG

Protein Name: Lysosomal alpha-glucosidase

Poids moléculaire: 98kDa

ID gène: 2548

NCBI Accession: NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process