Hexosaminidase A Antikörper

Produktnummer ABIN1846273

Produktdetails anti-Hexosaminidase A Antikörper

Target: Hexosaminidase A (HEXA)

Reaktivität: Human, Maus, Ratte

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Hexosaminidase A Antikörper ist unkonjugiert

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

Aufreinigung: Purified antibody

Immunogen: Purified recombinant fragment of human HEXA expressed in E. coli.

Isotyp: IgG2b

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1.0 mg/mL

Buffer: PBS with 0.05 % sodium azide and 0.5 % protein stabilizer.

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Informationen zur Lagerung: Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles.

Details zu Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Andere Bezeichnung: HEXA (HEXA Produkte)

Hintergrund: This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes.Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases.Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses.Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).

Molekulargewicht: 60.7 kDa

Gen-ID: 3073, 15211, 300757

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process