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Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
It is shown that TDP-43 is the major disease protein in both frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis.
Alpha-synuclein in Lewy bodies.
Diagnosis and management of dementia with Lewy bodies
The authors propose a new scheme for the pathologic assessment of LBs and Lewy neurites (LN) using alpha-synuclein immunohistochemistry and semiquantitative grading of lesion density, with the pattern of regional involvement being more important than total LB count.
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
It is shown that ataxin 2 (ATXN2), a polyglutamine (polyQ) protein mutated in spinocerebellar ataxia type 2, is a potent modifier of TDP-43 toxicity in animal and cellular models.
α-Synuclein in Lewy bodies
- M. Spillantini, M. L. Schmidt, V. Lee, J. Trojanowski, R. Jakes, M. Goedert
- 28 August 1997
Strong staining of Lewy bodies from idiopathic Parkinson's disease with antibodies for α-synuclein, a presynaptic protein of unknown function which is mutated in some familial cases of the disease, indicates that the LewY bodies from these two diseases may have identical compositions.
Synapse Loss and Microglial Activation Precede Tangles in a P301S Tauopathy Mouse Model
Tau-mediated neurodegeneration in Alzheimer's disease and related disorders
This Review summarizes the most recent advances in knowledge of the mechanisms of tau-mediated neurodegeneration to forge an integrated concept of those t Tau-linked disease processes that drive the onset and progression of AD and related tauopathies.
Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein.
It is demonstrated that A53T alpha-synuclein leads to the formation of toxic filamentous alpha- Synuclein neuronal inclusions that cause neurodegeneration.
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration
The aim of this study was to improve the neuropathologic recognition and provide criteria for the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal lobar degeneration (FTLD) and incorporate up-to-date neuropathology in the light of recent immunohistochemical, biochemical, and genetic advances.