Bu, Bitao and Kluenemann, Hans and Suzuki, Kinuko and Li, Jin and Bird, Thomas and Jin, Lee-Way and Vincent, Inez (2002) Niemann-Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles. NEUROBIOLOGY OF DISEASE, 11 (2). pp. 285-297. ISSN 0969-9961
Full text not available from this repository.Abstract
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 display several early markers of NFT formation, i.e., hyperphosphorylated tau and an array of cell cycle regulators, suggesting that cerebellar neurons in NPC undergo similar modifications as other neurons that develop NFT. However, cerebellar neurons are deficient in tau, the building block of NFT, and this may be one reason for their inability to form NFT. Even without NFT, cerebellar neurodegeneration may be triggered by the inappropriate activation of the cell cycle cdc2 kinase, and the npc-1 murine model provides an opportunity to test this hypothesis. (C) 2002 Elsevier Science (USA).
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | PAIRED HELICAL FILAMENTS; MOLECULAR-WEIGHT TAU; ALZHEIMERS-DISEASE; NERVOUS-SYSTEM; TYROSINE PHOSPHATASE; PROTEIN-TAU; BRAIN; PHOSPHORYLATION; ALZ-50; MOUSE; neurofibrillary tangle; neurodegeneration; Niemann-Pick disease type C; Alzheimer's disease; cerebellum; Purkinje neurons; hyperphosphorylated tau; cell cycle; cdc2 |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Psychiatrie und Psychotherapie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 27 Aug 2021 04:38 |
| Last Modified: | 27 Aug 2021 04:38 |
| URI: | https://pred.uni-regensburg.de/id/eprint/39750 |
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