Hexosaminidase A Antikörper (AA 89-529)

Produktnummer ABIN5540052

Der Maus Monoklonal anti-Hexosaminidase A Antikörper wird verwendet zum Nachweis von Hexosaminidase A in Proben von Human. Er wurde validiert für WB, FACS und EIA.

Kurzübersicht für Hexosaminidase A Antikörper (AA 89-529) (ABIN5540052)

Target: Hexosaminidase A (HEXA)

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Hexosaminidase A Antikörper ist unkonjugiert

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

Klon: AT20F1

Produktdetails anti-Hexosaminidase A Antikörper

Bindungsspezifität: AA 89-529

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

Aufreinigung: Protein-A affinity chromatography

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

Isotyp: IgG2a

Anwendungsinformationen

Applikationshinweise: ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

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

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Haltbarkeit: 12 months

Details zu Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Andere Bezeichnung: beta-hexosaminidase alpha,hexa

Hintergrund: 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