Optimal working dilution should be determined by the investigator.
Beschränkungen
Nur für Forschungszwecke einsetzbar
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.
Target
Hexosaminidase A (HEXA)
Andere Bezeichnung
HEXA
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).