Hexosaminidase A antibody (Middle Region)

Catalog No. ABIN3031197

Product Details anti-Hexosaminidase A Antibody

Target: Hexosaminidase A (HEXA)

Binding Specificity: Middle Region

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Hexosaminidase A antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Purification: Antigen affinity

Immunogen: An amino acid sequence from the middle region of human Hexosaminidase A, alpha subunit (DVMAYNKLNVFHWHLVD) was used as the immunogen for this HEXA antibody.

Isotype: IgG

Application Details

Application Notes: 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,IHC (Paraffin): 0.5-1 μg/mL

Restrictions: For Research Use only

Handling

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

Storage: -20 °C

Storage Comment: 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.

Target Details for Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Alternative Name: HEXA (HEXA Products)

Synonyms: TSD antibody, fc04h11 antibody, wu:fc04h11 antibody, wu:fv09f06 antibody, si:dkey-35i22.2 antibody, HEXA antibody, Hex-1 antibody, zgc:112084 antibody, hexosaminidase subunit alpha antibody, hexosaminidase B (beta polypeptide) antibody, CUGBP Elav-like family member 6 antibody, hexosaminidase A antibody, hexosaminidase A (alpha polypeptide) antibody, beta-hexosaminidase subunit alpha-like antibody, HEXA antibody, hexb antibody, CELF6 antibody, Hexa antibody, hexa antibody, LOC100228061 antibody

Background: HEXA (Hexosaminidase A) 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. 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 two 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