Expression of ubiquitin-binding protein p62 in ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis with dementia: analysis of five autopsy cases with broad clinicopathological spectrum

摘要: Amyotrophic lateral sclerosis with dementia (ALSD), corresponding to the motor neuron disease type of frontotemporal dementia, is neuropathologically characterized by depletion neurons, degeneration extra-motor cerebral cortices and formation ubiquitin-immunoreactive (not argyrophilic, tau-negative, α-synuclein-negative) intraneuronal inclusions. Recently, immunoreactivity for ubiquitin-binding protein p62 has been reported in several ubiquitin-containing or intraglial inclusions (e.g. neurofibrillary tangles, Pick bodies, Lewy glial cytoplasmic inclusions) various neurodegenerative diseases. We examined five ALSD cases a broad clinicopathological spectrum. was seen all cases. The mean proportion p62-immunoreactive total number (p62/Ub ratio) dentate gyrus 27.5±16.6% (range 6.3–47.3%). There no correlation between p62/Ub ratio severity duration illness neuropathological severity. Although main constituent these unknown, our study suggests that contributes via same mechanism as other previously Since believed have neuroprotective role, may represent non-harmful, rather protective effect against neuronal ALSD.