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Active Hexosaminidase A Alpha (HEXa)

TSD; Hex-A; Tay Sachs Disease; GM2 Gangliosidosis; Beta-Hexosaminidase Subunit Alpha; Beta-N-Acetylhexosaminidase Subunit Alpha; N-Acetyl-Beta-Glucosaminidase Alpha

ACTIVITY TEST

Hexosaminidase A Alpha (HEXa) is a lysosomal enzyme. There are three predominant isoenzymes: hexosaminidase A, B and S. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. The enzymes are composed of two alpha and/or beta subunits, which are coded by HEXA and HEXB genes, respectively. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit which contains a key residue, Arg-424 is able to hydrolyze GM2 gangliosides. Hexosaminidase A (alpha polypeptide) plays a critical role in the brain and spinal cord (central nervous system). Besides, Hexosaminidase B Beta (HEXB) has been identified as an interactor of HEXA, thus a binding ELISA assay was conducted to detect the interaction of recombinant mouse HEXA and recombinant mouse HEXB. Briefly, HEXA were diluted serially in PBS, with 0.01% BSA (pH 7.4). Duplicate samples of 100uL were then transferred to HEXB-coated microtiter wells and incubated for 2h at 37℃. Wells were washed with PBST and incubated for 1h with anti-HEXA pAb, then aspirated and washed 3 times. After incubation with HRP labelled secondary antibody, wells were aspirated and washed 3 times. With the addition of substrate solution, wells were incubated 15-25 minutes at 37℃. Finally, add 50µL stop solution to the wells and read at 450nm immediately. The binding activity of of HEXA and HEXB was shown in Figure 1, and this effect was in a dose dependent manner.

USAGE

Reconstitute in 20mM Tris, 150mM NaCl (pH8.0) to a concentration of 0.1-1.0 mg/mL. Do not vortex.

STORAGE

Avoid repeated freeze/thaw cycles. Store at 2-8°C for one month. Aliquot and store at -80°C for 12 months.

STABILITY

The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

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Magazine Citations
Neural Regen Res Functionality of a bicistronic construction containing HEXA and HEXB genes encoding ¦Â-hexosaminidase A for cell-mediated therapy of GM2 gangliosidoses 34100447
life Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM2 Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell … 34685379
Catalog No. Related products for research use of Mus musculus (Mouse) Organism species Applications (RESEARCH USE ONLY!)
APA195Mu01 Active Hexosaminidase A Alpha (HEXa) Cell culture; Activity Assays.
RPA195Mu01 Recombinant Hexosaminidase A Alpha (HEXa) Positive Control; Immunogen; SDS-PAGE; WB.
RPA195Mu02 Recombinant Hexosaminidase A Alpha (HEXa) Positive Control; Immunogen; SDS-PAGE; WB.
PAA195Mu01 Polyclonal Antibody to Hexosaminidase A Alpha (HEXa) WB; IHC; ICC; IP.
PAA195Mu02 Polyclonal Antibody to Hexosaminidase A Alpha (HEXa) WB; IHC; ICC; IP.
SEA195Mu ELISA Kit for Hexosaminidase A Alpha (HEXa) Enzyme-linked immunosorbent assay for Antigen Detection.
LMA195Mu Multiplex Assay Kit for Hexosaminidase A Alpha (HEXa) ,etc. by FLIA (Flow Luminescence Immunoassay) FLIA Kit for Antigen Detection.