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Valosin‐containing protein immunoreactivity in tauopathies,synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease
Authors:Fumiaki Mori  Kunikazu Tanji  Yasuko Toyoshima  Hidenao Sasaki  Mari Yoshida  Akiyoshi Kakita  Hitoshi Takahashi  Koichi Wakabayashi
Affiliation:1. Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, , Hirosaki, Japan;2. Department of Pathology, University of Niigata, , Niigata, Japan;3. Department of Neurology, Hokkaido University Graduate School of Medicine, , Sapporo, Japan;4. Department of Neuropathology, Aichi Medical University, , Nagakute, Japan;5. Department of Pathological Neuroscience, Center for Bioresource‐Based Researches, Brain Research Institute, University of Niigata, , Niigata, Japan
Abstract:Valosin‐containing protein (VCP) is associated with multiple cellular functions, including ubiquitin‐dependent protein degradation. Mutations in VCP are known to cause inclusion body myopathy with Paget's disease and frontotemporal dementia and familial amyotrophic lateral sclerosis (fALS; ALS14), both of which are characterized by trans‐activation response DNA protein 43 (TDP‐43)‐positive neuronal cytoplasmic and nuclear inclusions. Recently, immunoreactivity for fALS‐associated proteins (TDP‐43, fused in sarcoma (FUS), optineurin and ubiquilin‐2) were reported to be present in cytoplasmic and nuclear inclusions in various neurodegenerative diseases. However, the extent and frequency of VCP‐immunoreactive structures in these neurodegenerative diseases are uncertain. We immunohistochemically examined the brains of 72 cases with neurodegenerative diseases and five control cases. VCP immunoreactivity was present in Lewy bodies in Parkinson's disease and dementia with Lewy bodies, and neuronal nuclear inclusions in five polyglutamine diseases and intranuclear inclusion body disease, as well as in Marinesco bodies in aged control subjects. However, other neuronal and glial cytoplasmic inclusions in tauopathies and TDP‐43 proteinopathies were unstained. These findings suggest that VCP may have common mechanisms in the formation or degradation of cytoplasmic and nuclear inclusions of neurons, but not of glial cells, in several neurodegenerative conditions.
Keywords:Lewy body  Marinesco body  neuronal nuclear inclusion  polyglutamine disease  valosin‐containing protein
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