HEXB Antikörper

Produktnummer ABIN7881035

Dieser Maus Monoklonal Antikörper detektiert spezifisch HEXB in IHC (p). Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für HEXB Antikörper (ABIN7881035)

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))

Klon: HEXB-7762

Produktdetails anti-HEXB Antikörper

Verwendungszweck: Hexosaminidase B Antibody / HEXB

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: 0.2 mg/mL

Buffer: 0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05 % sodium azide

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 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