Hexosaminidase A anticorps (AA 89-529)

N° du produit ABIN5775909

Cet anticorps Souris Monoclonal détecte spécifiquement Hexosaminidase A dans WB, ELISA et FACS. Il présente une réactivité envers Humain.

Aperçu rapide pour Hexosaminidase A anticorps (AA 89-529) (ABIN5775909)

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp Hexosaminidase A est non-conjugé

Application: Western Blotting (WB), ELISA, Flow Cytometry (FACS)

Clone: AT20F1

Détail du produit anti-Hexosaminidase A anticorps

Épitope: AA 89-529

Fonction: Human Hexosaminidase A/HEXA antibody

Purification: Purified

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

Isotype: IgG2a lambda

Information d'application

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

Buffer: Phosphate-Buffered Saline ( pH 7.4) with 0.02 % Sodium Azide, 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,-80 °C

Stockage commentaire: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: Hexosaminidase A/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.

NCBI Accession: NP_000511

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process