Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology

Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (

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Bibliographic Details
Published in:Acta neuropathologica 2009, Vol.117 (1), p.45-53
Main Authors: Nishihira, Yasushi, Tan, Chun-Feng, Hoshi, Yasuhiro, Iwanaga, Keisuke, Yamada, Megumi, Kawachi, Izumi, Tsujihata, Mitsuhiro, Hozumi, Isao, Morita, Takashi, Onodera, Osamu, Nishizawa, Masatoyo, Kakita, Akiyoshi, Takahashi, Hitoshi
Format: Article
Language:English
Subjects:
Aged
Alzheimer's disease
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - metabolism
Amyotrophic Lateral Sclerosis - pathology
Anterior Horn Cells - metabolism
Anterior Horn Cells - pathology
Anterior Horn Cells - ultrastructure
Autopsies
Autopsy
Brain - metabolism
Brain - pathology
Brain research
Brain Stem - metabolism
Brain Stem - pathology
Cerebrum - metabolism
Cerebrum - pathology
Dementia
Dentate Gyrus - metabolism
Dentate Gyrus - pathology
Disease
DNA-Binding Proteins - metabolism
Dysphagia
Dyspnea
Female
Hippocampus - metabolism
Hippocampus - pathology
Humans
Immunohistochemistry
Inclusion Bodies - metabolism
Inclusion Bodies - pathology
Male
Medicine
Medicine & Public Health
Microscopy, Immunoelectron
Middle Aged
Nervous system
Neuroglia - metabolism
Neuroglia - pathology
Neurology
Neurons
Neurons - metabolism
Neurons - pathology
Neurosciences
Original Paper
Pathology
Research centers
Spinal Cord - metabolism
Spinal Cord - pathology
Substantia Nigra - metabolism
Substantia Nigra - pathology
Time Factors
Ubiquitin - metabolism
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Description
Summary:Recently, sporadic amyotrophic lateral sclerosis (SALS), a fatal neurological disease, has been shown to be a multisystem proteinopathy of TDP-43 in which both neurons and glial cells in the central nervous system are widely affected. In general, the natural history of SALS is short (
ISSN:0001-6322
1432-0533
DOI:10.1007/s00401-008-0443-6