Article
Niemann-Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles.
Neurobiology of disease - 1 Nov 2002
Bu Bitao, Klunemann Hans, Suzuki Kinuko, Li Jin, Bird Thomas, Jin Lee-Way, Vincent Inez
Abstract excerpt
It is unknown why cerebellar neurons resist neurofibrillary tangle (NFT) formation. In Niemann-Pick disease Type C (NPC), NFT-mediated neurodegeneration occurs throughout brain, but the cerebellum degenerates conspicuously without NFT. To understand why, we have studied markers of NFT pathogenesis in cerebellum from 17 NPC cases, all having abundant NFT in forebrain. Remarkably, we found that NPC cerebella...
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