Selective degeneration fo Purkinje cells with Lewy body-like inclusions in aged NFHLACZ transgenic mice.

摘要: Transgenic (NFHLacZ) mice expressing a fusion protein composed of truncated high-molecular-weight mouse neurofilament (NF) (NFH) fused to beta-galactosidase (LacZ) develop inclusions in neurons throughout the CNS. These persist from birth advanced age and contain massive filamentous aggregates including all three endogenous NF proteins NFHLacZ protein. Further, levels are selectively reduced mice. Because these resemble NF-rich Lewy bodies (LBs) Parkinson's disease LB dementia, we asked whether lesions compromised viability affected during aging. We studied hippocampal CA1 neurons, nearly which harbored (type I) devoid cellular organelles, cerebellar Purkinje cells, accumulated II) containing numerous entrapped organelles. cells with type II began degenerate at approximately 1 year age, most were eliminated by 18 months age. In contrast, there was no significant loss I inclusion-bearing data suggest that sequestration organelles may isolate impair function thereby rendering vulnerable degeneration as neurodegenerative diseases elderly characterized accumulation LBs.