This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Pathology
[{'content': "Deficiency of enzyme hexosaminidase, subunit causes - \nSelect and explain. \nA. Tay-Sachs disease, B. Hurler's syndrome, C. Fabry disease, D. Pompe disease", 'role': 'user'}, {'content': 'Answer: A \nExplanation: Tay-Sachs Disease (GM2 Gangliosidosis: Deficiency in Hexosaminidase β Subunit) Gangliosidoses are characterized by the accumulation of gangliosides, principally in the brain, as a result of a deficiency of a catabolic lysosomal enzyme.\nDepending on the ganglioside involved, these disorders are subclassified into GM1 and GM2 categories. Tay-Sachs disease, by far the most common of all gangliosidoses, is characterized by a mutation in and consequent deficiency of the β subunit of enzyme hexosaminidase A, which is necessary for the degradation of GM2. More than 100 mutations have been described; most affect protein folding or intracellular transport.', 'role': 'assistant'}]