Hexosaminidase A anticorps (AA 89-529)

N° du produit ABIN5540052

L’anticorps anti-Hexosaminidase A Monoclonal Souris est utilisé pour la détection de Hexosaminidase A dans des échantillons de Humain. Il a été validé pour WB, FACS et EIA.

Aperçu rapide pour Hexosaminidase A anticorps (AA 89-529) (ABIN5540052)

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp Hexosaminidase A est non-conjugé

Application: Western Blotting (WB), Flow Cytometry (FACS), Enzyme Immunoassay (EIA)

Clone: AT20F1

Détail du produit anti-Hexosaminidase A anticorps

Épitope: AA 89-529

Specificité: This antibody detects HEXA at aa 89-529.

Purification: Protein-A affinity chromatography

Immunogène: Recombinant human HEXA (89-529aa) purified from E. coli

Isotype: IgG2a

Information d'application

Indications d'application: ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: PBS, pH 7.4 containing 0.02 % Sodium Azide and 10 % Glycerol

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 undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

Date de péremption: 12 months

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: beta-hexosaminidase alpha,hexa

Sujet: HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process