Hexosaminidase A anticorps (C-Term)

N° du produit ABIN3031198

Cet anticorps Lapin Polyclonal détecte spécifiquement Hexosaminidase A dans WB. Il présente une réactivité envers Humain.

Aperçu rapide pour Hexosaminidase A anticorps (C-Term) (ABIN3031198)

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Hexosaminidase A est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-Hexosaminidase A anticorps

Épitope: C-Term

Purification: Antigen affinity

Immunogène: An amino acid sequence from the C-terminus of human HEXA (QAQPLNVGFCEQEFEQT) was used as the immunogen for this HEXA antibody.

Isotype: IgG

Information d'application

Indications d'application: The stated application concentrations are suggested starting amounts. Titration of the HEXA antibody may be required due to differences in protocols and secondary/substrate sensitivity.\. Western blot: 0.5-1 μg/mL

Restrictions: For Research Use only

Stockage

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Stock: -20 °C

Stockage commentaire: After reconstitution, the HEXA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: HEXA

Sujet: Hexosaminidase A (alpha polypeptide) is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene encodes the alpha subunit of hexosaminidase A, a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit(amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process