Hexosaminidase A anticorps (C-Term) (Biotin)

N° du produit ABIN7997497

L’anticorps anti-Hexosaminidase A Polyclonal Lapin est utilisé pour la détection de Hexosaminidase A dans des échantillons de Humain. Il a été validé pour WB, ELISA et IHC.

Aperçu rapide pour Hexosaminidase A anticorps (C-Term) (Biotin) (ABIN7997497)

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Hexosaminidase A est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (IHC)

Détail du produit anti-Hexosaminidase A anticorps (Biotin)

Épitope: C-Term

Fonction: Anti-HEXA Antibody Biotin Conjugated

Specificité: No cross reactivity with other proteins.

Réactivité croisée (Details): No cross-reactivity with other proteins

Homologie: different from the related mouse sequence by three amino acids,and from the related rat sequences by four amino acids.

Purification: Immunogen affinity purified.

Immunogène: A synthetic peptide corresponding to a sequence at the C-terminus of human HEXA, different from the related mouse sequence by three amino acids, and from the related rat sequences by four amino acids.

Isotype: IgG

Information d'application

Indications d'application: Western blot, Optimal dilutions should be determined by end users. Immunohistochemistry (Paraffin-embedded Section), Optimal dilutions should be determined by end users. ELISA, Optimal dilutions should be determined by end users.

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % 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: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing.

Date de péremption: 12 months

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: HEXA

Sujet:

Background: HEXA (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.

Gene Full Name: hexosaminidase subunit alpha

Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.

ID gène: 3073

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process