HEXA also known as TSD is a member of the glycosyl hydrolase 20 family of proteins. HEXA plays a vital role in the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines. Proper functioning of HEXA is essential for maintaining cellular health, as HEXA deficiency leads to the accumulation of GM2 gangliosides, which is a hallmark of Tay-Sachs disease (TSD), a severe autosomal recessive lysosomal storage disorder affecting the central nervous system. TSD arises from mutations in the HEXA gene, resulting in insufficient HEXA enzyme activity and subsequent neurodegeneration due to the toxic buildup of GM2 gangliosides.