Mutant ubiquitin and p62 immunoreactivity in cases of combined multiple system atrophy and Alzheimer’s disease
| Autor: | Isidro Ferrer, Beatrice Terni, Fred W. van Leeuwen, Benjamín Torrejón-Escribano, Matil Calopa, M. Pujol Sabate, María Jesús Rey, Susana Boluda |
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| Rok vydání: | 2007 |
| Předmět: |
Male
Sequestosome-1 Protein Pathology medicine.medical_specialty Cytoplasmic inclusion Hippocampus tau Proteins Hippocampal formation Pathology and Forensic Medicine Cellular and Molecular Neuroscience Atrophy Ubiquitin Alzheimer Disease mental disorders medicine Humans Adaptor Proteins Signal Transducing Aged biology Neurofibrillary Tangles Multiple System Atrophy Entorhinal cortex medicine.disease nervous system diseases nervous system Proteasome alpha-Synuclein biology.protein Neurology (clinical) |
| Zdroj: | Acta Neuropathologica. 113:403-416 |
| ISSN: | 1432-0533 0001-6322 |
| DOI: | 10.1007/s00401-006-0192-3 |
| Popis: | Recent studies have shown the co-existence of alpha-synuclein and phosphorylated tau (pTau) in several neurodegenerative diseases. Here, we report two autopsy cases of combined multiple system atrophy (MSA) and Alzheimer's disease (AD). In both cases, abundant alpha-synuclein-positive glial and neuronal cytoplasmic inclusions were found in the brainstem, amygdala and hippocampal formation. pTau-positive neurofibrillary tangles (NFTs) were widely distributed in case 1 (Braak stage VI) and moderate in case 2 (Braak stage III). Although alpha-synuclein and pTau pathology co-occurred in the hippocampus and entorhinal cortex, only a few neurons showed co-existence of these two proteins. Immunoreactivity for p62, a ubiquitin proteasome system related protein, was found in the majority of NFTs, but in only a small proportion of neuronal alpha-synuclein inclusions. In addition, UBB+1, a mutant form of ubiquitin and a marker for proteasomal dysfunction, was present in the majority of NFTs, whereas co-existence of alpha-synuclein and UBB+1 was found in only a few neurons. These findings indicate that alpha-synuclein and phosphorylated tau co-occur in certain brain regions in cases of combined MSA and AD and that the proteasomal pathways differ between alpha-synuclein- and pTau-bearing neurons. |
| Databáze: | OpenAIRE |
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