Article
Establishment and characterization of immortalized Schwann cells from murine model of Niemann-Pick disease type C (spm/spm).
Journal of the peripheral nervous system : JPNS - 1 Jun 2001
Watabe K, Ida H, Uehara K, Oyanagi K, Sakamoto T, Tanaka J, Garver W S, Miyawaki S, Ohno K, Eto Y
Abstract excerpt
Niemann-Pick disease type C (NPC) is characterized by an accumulation of unesterified cholesterol in the endosomal/lysosomal (E/L) system, resulting in progressive neurodegeneration and death during early childhood. To investigate the cellular pathomechanism of nervous system involvement in NPC,...
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