GANGLIOSIDOSIS
\ɡˌaŋɡlɪˌɒsɪdˈə͡ʊsɪs], \ɡˌaŋɡlɪˌɒsɪdˈəʊsɪs], \ɡ_ˌa_ŋ_ɡ_l_ɪ__ˌɒ_s_ɪ_d_ˈəʊ_s_ɪ_s]\
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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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Platidiam
- An inorganic water-soluble platinum complex. After undergoing hydrolysis, it reacts DNA produce both intra interstrand crosslinks. These crosslinks appear to impair replication and transcription of DNA. The cytotoxicity cisplatin correlates with cellular arrest in G2 phase cell cycle.