GAA anticorps (Middle Region)

N° du produit ABIN4886596

Cet anticorps Lapin Polyclonal détecte spécifiquement GAA dans WB et IHC. Il présente une réactivité envers Humain.

Aperçu rapide pour GAA anticorps (Middle Region) (ABIN4886596)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GAA est non-conjugé

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

Détail du produit anti-GAA anticorps

Épitope: AA 494-527, Middle Region

Fonction: Anti-GAA Antibody Picoband®

Séquence: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

Réactivité croisée (Details): No cross-reactivity with other proteins

Attributs du produit: Anti-GAA Antibody Picoband® (ABIN4886596). Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Purification: Immunogen affinity purified.

Immunogène: A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Isotype: IgG

Information d'application

Indications d'application: Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
Western blot, 0.1-0.5 μg/mL, Human
1. "Entrez Gene: GAA glucosidase, alpha, acid (Pompe disease, glycogen storage disease type II)". 2. Donald J. Voet, Judith G. Voet, Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61-9.

Commentaires:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg 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.

Conseil sur la manipulation: Avoid repeated freezing and thawing.

Stock: 4 °C,-20 °C

Stockage commentaire: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Détails sur GAA

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

Autre désignation: GAA

Sujet:

Synonyms: Lysosomal alpha-glucosidase,3.2.1.20,Acid maltase,Aglucosidase alfa,76 kDa lysosomal alpha-glucosidase,70 kDa lysosomal alpha-glucosidase,GAA,

Tissue Specificity: Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

Background: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

Poids moléculaire: 110 kDa, 95kDa, 76kDa, 70 kDa

ID gène: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process