HEXB Antikörper

Produktnummer ABIN7864758

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

Kurzübersicht für HEXB Antikörper (ABIN7864758)

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

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser HEXB Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (IHC)

Klon: HEXB-7762

Produktdetails anti-HEXB Antikörper

Verwendungszweck: HEXB Antibody

Spezifität: Hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a tetramer of two ≤-A and two ≤-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 ≤ 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.

Aufreinigung: 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.

Immunogen: Recombinant fragment human HEXB protein (exact sequence is proprietary)

Isotyp: IgG2, kappa

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 200 μg/mL

Buffer: Prepared in 10 mM PBS with 0.05 % BSA & 0.05 % azide.

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

Informationen zur Lagerung: Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C.Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Details zu HEXB

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

Andere Bezeichnung: HEXB

Hintergrund:

Beta N acetylhexosaminidase, Cervical cancer proto oncogene 7 protein, ENC 1AS, Epididymis luminal protein 248, HCC7, HEL 248, Hexosaminidase B (beta polypeptide), Hexosaminidase B, N-acetyl-beta-glucosaminidase subunit beta

Human kidney or lung.

Molekulargewicht: 63kDa

Gen-ID: 3074, 69293

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process