Hexosaminidase A Antikörper (Middle Region)

Produktnummer ABIN3044173

Produktdetails anti-Hexosaminidase A Antikörper

Target: Hexosaminidase A (HEXA)

Bindungsspezifität: AA 191-207, Middle Region

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser Hexosaminidase A Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC)

Verwendungszweck: Anti-HEXA Antibody Picoband®

Sequenz: DVMAYNKLNV FHWHLVD

Kreuzreaktivität (Details): No cross-reactivity with other proteins

Produktmerkmale: Anti-HEXA Antibody (ABIN3044173). Tested in IHC, 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.

Aufreinigung: Immunogen affinity purified.

Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human HEXA, different from the related rat and mouse sequences by one amino acid.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human, Rat, Mouse
Western blot, 0.1-0.5 μg/mL, Human, Rat, Mouse
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.

Kommentare:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

Buffer: Each vial contains 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Thimerosal, 0.05 mg Sodium azide.

Konservierungsmittel: Thimerosal (Merthiolate), Sodium azide

Vorsichtsmaßnahmen: This product contains Thimerosal (Merthiolate) and Sodium azide: POISONOUS AND HAZARDOUS SUBSTANCES which should be handled by trained staff only.

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Store 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 freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Andere Bezeichnung: HEXA (HEXA Produkte)

Hintergrund:

Synonyms: Beta-hexosaminidase subunit alpha,3.2.1.52,Beta-N-acetylhexosaminidase subunit alpha,Hexosaminidase subunit A,N-acetyl-beta-glucosaminidase subunit alpha,HEXA,

Tissue Specificity: Expressed in various epithelial tissues. Abundantly expressed in liver and kidney, and to a lesser extent in lung. The tissue distribution of isoforms 1 and 1B is distinct. .

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.

Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.

Molekulargewicht: 61 kDa

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process