HEXA (Beta-N-acetylhexosaminidase subunit alpha)

Size

£620.00

SKU:: 144657
Purity:: Purified by immunoaffinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to aa191-207, DVMAYNKLNVFHWHLVD, in the middle region of human HEXA, different from the related rat and mouse sequences by one amino acid. UniProt: P06865 Species Sequence Homology: mouse
Format:: Supplied as a lyophilized powder from PBS, 5% BSA, 0.05% thimerosal, 0.05% sodium azide. Reconstitute with 200ul sterile ddH2O.
Extra Details:: 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 tohydrolyze 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. Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilutions: Western Blot: 01.-0.5ug/ml Immunohistochemistry (FFPE): 0.5-1ug/ml. Boiling the paraffin sections in 10mM citrate buffer, pH 6.0, for 20 minutes is required for the staining of formalin/paraffin sections. Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized powder may be stored at -20°C. Stable for 12 months after receipt at -20°C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Shipping Conditions:: Blue Ice