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alpha autosomal betahexosaminidase brain cell chemical congenital deficiency demyelination disease disorder dna enzyme ganglioside gangliosidosis gene genetic gm2 hexa hexosaminidase human illustration infancy infant inherited lamellar lipid lysosomal lysosome medical membraneous metabolic molecular molecule mutation neurology neuron ocular pathology polypeptide rare recessive science sphingolipidosis storage structure taysachs tsd