H2047-02A

HEXA (Beta-hexosaminidase Subunit alpha, Beta-N-acetylhexosaminidase Subunit alpha, Hexosaminidase Subunit A, N-acetyl-beta-glucosaminidase Subunit alpha)

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£832.00

SKU:: H2047-02A
Purity:: Purified by Protein A affinity chromatography from hybridoma culture supernatant.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Recombinant protein corresponding to Met1-Thr529 from human HEXA, expressed in Sf21 cell line.
Clone:: 12K101
Format:: Supplied as a lyophilized powder from PBS, pH 7.4, 5% trehalose. Reconstitute with 200ul sterile PBS.
Extra Details:: B-hexosaminidases are enzymes involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo-sphingolipids in lysosomes. The enzymes are composed of two A and/or B subunits, which are coded by HEXA and HEXB genes, respectively. Different association of the A and B subunits gives rise to B-hexosaminidase isoforms A, B and S (Hex A, B and S), which have the composition of AB, BB and AA respectively. Our recombinant HEXA is presumably isoform Hex S, because only A subunit was expressed. Hex S is suggested to releases non-reducing end N-acetylgalactosamine residues from dermatan sulfate, chondroitin sulfate and sulfated glycolipid SM2. Recombinant HEXA is also highly active on 4-methylumbelliferyl-N-acetyl -B-D-glucosaminide. Mutations in HEXA and HEXB genes cause lysosomal lipid storage disorders. Specifically, mutations of HEXA cause Tay-Sachs disease, manifested by the harmful accumulation of ganglioside GM2 in tissues and nerve cells in the brain. Children with this disease usually die by age 4. Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilutions: Western Blot: 2ug/ml using HepG2 human hepatocellular carcinoma cell line and human liver tissue Immunohistochemistry: 8-25ug/ml using immersion fixed paraffin-embedded sections of human brain (hypothalamus) Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C. Reconstitute with sterile PBS. 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