GANGLIOSIDOSES
\ɡˈaŋɡlɪˌɒsɪdˌə͡ʊzɪz], \ɡˈaŋɡlɪˌɒsɪdˌəʊzɪz], \ɡ_ˈa_ŋ_ɡ_l_ɪ__ˌɒ_s_ɪ_d_ˌəʊ_z_ɪ_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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Word of the day
Dopamine Acetyltransferase
- An enzyme that catalyzes the of groups from acetyl-CoA to arylamines. They have wide specificity for aromatic amines, particularly serotonin, and can also catalyze acetyl transfer between arylamines without CoA. EC 2.3.1.5.