HEXB anticorps

N° du produit ABIN7881035

Cet anticorps Souris Monoclonal détecte spécifiquement HEXB dans IHC (p). Il présente une réactivité avec des échantillons de Humain.

Aperçu rapide pour HEXB anticorps (ABIN7881035)

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

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp HEXB est non-conjugé

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

Clone: HEXB-7762

Détail du produit anti-HEXB anticorps

Fonction: Hexosaminidase B Antibody / HEXB

Purification: Protein A/G affinity

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

Isotype: IgG2, kappa

Information d'application

Indications d'application: Optimal dilution of the Hexosaminidase B antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.2 mg/mL

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

Agent conservateur: Sodium azide

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

Stock: -20 °C

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

Détails sur HEXB

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

Autre désignation: Hexosaminidase B

Sujet: 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