GANGLIOSIDE STORAGE DISEASE
\ɡˈaŋɡlɪˌɒsa͡ɪd stˈɔːɹɪd͡ʒ dɪzˈiːz], \ɡˈaŋɡlɪˌɒsaɪd stˈɔːɹɪdʒ dɪzˈiːz], \ɡ_ˈa_ŋ_ɡ_l_ɪ__ˌɒ_s_aɪ_d s_t_ˈɔː_ɹ_ɪ_dʒ d_ɪ_z_ˈiː_z]\
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A group of often fatal inherited diseases marked by the accumulation of GANGLIOSIDES in LYSOSOMES secondary to enzymatic deficiency states. Gangliosidoses include TAY- SACHS DISEASE; GANGLIOSIDOSIS GM1; GANGLIOSIDOSES GM2; and SANDHOFF DISEASE; which share the infantile or childhood onset of CENTRAL NERVOUS SYSTEM deterioration. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97)
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