GAA anticorps (N-Term)

N° du produit ABIN8101555

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB. Il présente une réactivité avec des échantillons de Humain.

Aperçu rapide pour GAA anticorps (N-Term) (ABIN8101555)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-GAA anticorps

Épitope: N-Term

Fonction: GAA Antibody - N-terminal region

Séquence: RDLAPTPGAN LYGSHPFYLA LEDGGSAHGV FLLNSNAMDV VLQPSPALSW

Homologie: Dog: 86%, Guinea Pig: 100%, Horse: 86%, Human: 100%, Mouse: 100%, Rabbit: 100%, Rat: 100%

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 AAP44227-100UG

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: 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.

Gene Name: glucosidase, alpha, acid

Alternative Symbols: LYAG

Protein Name: lysosomal alpha-glucosidase

Poids moléculaire: 104 kDa

ID gène: 2548

NCBI Accession: NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process