Tay-Sachs disease (MONDO_0010100, a Monarch Disease Ontology term) can be described as follows. GM2 gangliosidosis, variant B or Tay-Sachs disease is marked by accumulation of G2 gangliosides due to hexosaminidase A deficiency. Also known as: GM2 gangliosidosis, B, B1 variant, GM2-gangliosidosis, several forms, Hex A pseudodeficiency, Tay Sachs Disease, disease, Tay-Sachs, hexosaminidase A deficiency.