Hexosaminidase A anticorps (AA 135-179)

N° du produit ABIN7599980

Cet anticorps Lapin Polyclonal détecte spécifiquement Hexosaminidase A dans WB, ELISA, ICC et IF. Il présente une réactivité envers Humain, Souris et Rat.

Aperçu rapide pour Hexosaminidase A anticorps (AA 135-179) (ABIN7599980)

Antigène: Hexosaminidase A (HEXA)

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp Hexosaminidase A est non-conjugé

Application: Western Blotting (WB), ELISA, Immunocytochemistry (ICC), Immunofluorescence (IF)

Détail du produit anti-Hexosaminidase A anticorps

Épitope: AA 135-179

Fonction: Anti-HEXA Antibody Picoband®

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

Attributs du produit: Anti-HEXA Antibody Picoband® (ABIN7599980). Tested in ELISA, IF, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Purification: Immunogen affinity purified.

Immunogène: E.coli-derived human HEXA recombinant protein (Position: A135-H179).

Isotype: IgG

Information d'application

Indications d'application: Western blot, 0.1-0.25 μg/mL, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Akli, S., Chomel, J.-C., Lacorte, J.-M., Bachner, L., Poenaru, A., Poenaru, L. Ten novel mutations in the HEXA gene in non-Jewish Tay-Sachs patients. Hum. Molec. Genet. 2: 61-67, 1993. 2. Beutler, E., Kuhl, W., Comings, D. Hexosaminidase isozyme in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease). Am. J. Hum. Genet. 27: 628-638, 1975. 3. Chern, C. J., Beutler, E., Kuhl, W., Gilbert, F., Mellman, W. J., Croce, C. M. Characterization of heteropolymeric hexosaminidase A in human x mouse hybrid cells. Proc. Nat. Acad. Sci. 73: 3637-3640, 1976.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Stock: 4 °C,-20 °C

Stockage commentaire: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Détails sur Hexosaminidase A

Antigène: Hexosaminidase A (HEXA)

Autre désignation: HEXA

Sujet:

Synonyms: Early growth response protein 1

Tissue Specificity: Detected in neutrophils (at protein level).

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.

Poids moléculaire: 60 kDa

ID gène: 3073

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process