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A C57BL/KsJ mouse model of Niemann-Pick disease (spm) belongs to the same complementation group as the major childhood type of Niemann-Pick disease type C

✍ Scribed by S. Akaboshi; Tamami Yano; Shigeki Miyawaki; Kousaku Ohno; Kenzo Takeshita


Publisher
Springer
Year
1997
Tongue
English
Weight
111 KB
Volume
99
Category
Article
ISSN
0340-6717

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## Abstract Niemann‐Pick disease type C (NPC) is a deadly neurodegenerative disease often caused by mutation in a gene called NPC1, which results in the accumulation of unesterified cholesterol and glycosphingolipids in the endosomal–lysosomal system. Most studies on the mechanisms of neurodegenera