Human Hexosaminidase A Alpha (HEXa) ELISA Kit

Quantifying the alpha subunit of beta-hexosaminidase A, this in vitro research-use-only immunoassay kit from Assay Genie supports studies of lysosomal catabolism and GM2 ganglioside hydrolysis in human specimens. The HEXA gene product assembles with the HEXB-encoded beta subunit to form the active Hex A heterodimer, and altered expression has been implicated in Tay-Sachs disease, Sandhoff disease, and broader neurodegeneration research, alongside investigations into enzyme replacement therapies and substrate reduction approaches for lysosomal storage disorders.

The assay ships as a complete kit containing pre-coated microplate wells, lyophilized and liquid reagents, wash buffer concentrate, and a recombinant standard curve for interpolating unknown sample concentrations. Compatible input matrices include human blood-derived fractions, tissue homogenates prepared from cultured cells or biopsied material, and cell culture supernatant, making the workflow usable for cell-based assays and tissue lysate studies as well as standard biofluid workups. Storage temperature guidance applies to unopened kit components, with refrigerated handling of diluted working solutions and standards recommended to preserve antibody binding activity across the full run.

Applications span biomarker validation, longitudinal monitoring of enzyme levels in patient cohorts, and characterization of disease models, including iPSC-derived neuronal lines used to probe ganglioside accumulation and inflammation. Researchers focused on lysosomal storage disorders, neuroinflammation, and gene therapy development can use the kit to track treatment response and compare tissue-specific expression of the catalytic alpha subunit across experimental arms.