Hexosaminidase A Antikörper (AA 89-529)

Produktnummer ABIN5775909

Dieser Maus Monoklonal Antikörper erkennt spezifisch Hexosaminidase A in WB, ELISA und FACS. Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für Hexosaminidase A Antikörper (AA 89-529) (ABIN5775909)

Target: Hexosaminidase A (HEXA)

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Hexosaminidase A Antikörper ist unkonjugiert

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

Klon: AT20F1

Produktdetails anti-Hexosaminidase A Antikörper

Bindungsspezifität: AA 89-529

Verwendungszweck: Human Hexosaminidase A/HEXA antibody

Aufreinigung: Purified

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

Isotyp: IgG2a lambda

Anwendungsinformationen

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

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

Informationen zur Lagerung: 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.

Details zu Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Andere Bezeichnung: Hexosaminidase A/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.

NCBI Accession: NP_000511

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process