HEXB Antikörper

Produktnummer ABIN7881034

Der Maus Monoklonal anti-HEXB Antikörper (Klon HEXB-7762) (ABIN7881034) detektiert spezifisch HEXB in IHC (p). Dieser Antikörper reagiert spezifisch mit Proben aus Human.

Kurzübersicht für HEXB Antikörper (ABIN7881034)

Target: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser HEXB Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Güteklasse: Carrier-free

Klon: HEXB-7762

Produktdetails anti-HEXB Antikörper

Verwendungszweck: Hexosaminidase B Antibody / HEXB (azide and preservative free)

Aufreinigung: Protein A/G affinity

Immunogen: A recombinant fragment of the human protein was used as the immunogen for the Hexosaminidase B antibody.

Isotyp: IgG2, kappa

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the Hexosaminidase B antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

Buffer: 1 mg/mL in 1X PBS, BSA free, sodium azide free

Konservierungsmittel: Azide free

Lagerung: -20 °C

Informationen zur Lagerung: Aliquot the Hexosaminidase B antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Details zu HEXB

Target: HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))

Andere Bezeichnung: Hexosaminidase B

Hintergrund: Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a tetramer of two beta-A and two beta-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the beta subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.

UniProt: P07686

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process