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Optineurin is colocalized with ubiquitin in Marinesco bodies
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Open AccessDistinct glutaminyl cyclase expression in Edinger–Westphal nucleus, locus coeruleus and nucleus basalis Meynert contributes to pGlu-Aβ pathology in Alzheimer’s disease
Glutaminyl cyclase (QC) was discovered recently as the enzyme catalyzing the pyroglutamate (pGlu or pE) modification of N-terminally truncated Alzheimer’s disease (AD) Aβ peptides in vivo. This modification co...
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Article
TDP-43 pathology in familial British dementia
Trans-activation-responsive DNA-binding protein 43 (TDP-43) is a component of pathological inclusions in amyotrophic lateral sclerosis and several forms of sporadic and familial frontotemporal lobar degenerati...
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Inflammatory Processes Exacerbate Degenerative Neurological Disorders
Evidence that neuroinflammation exacerbates the pathology in Alzheimer’s disease (AD) has been accumulated from three independent fields of research: neuropathology demonstrating activated glial cells, epidemi...
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Article
Expression of BRI, the normal precursor of the amyloid protein of familial British dementia, in human brain
Familial British dementia (FBD) is characterized neuropathologically by deposition of a unique amyloid-forming protein, ABri. It is a fragment of an abnormal form of a precursor protein, BRI. In FBD, BRI is el...
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Article
Familial British dementia: colocalization of furin and ABri amyloid
Familial British dementia (FBD) is an autosomal dominant condition caused by a point mutation in the stop codon of the BRI gene. This mutation extends the normal precursor protein (PP) of 266 amino acids to the n...
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Article
Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with Pick's disease, corticobasal degeneration and progressive supranuclear palsy
We investigated isoform composition of aggregated tau protein in brains with Pick's disease (PiD), corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) by immunoblot analysis of sarkosyl-i...
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Article
Pyramidal neuron loss is matched by ghost tangle increase in Guam parkinsonism-dementia hippocampus
The parkinsonism dementia complex of Guam (bodig disease) is characterized by severe neurofibrillary tangle (NFT) development without the senile plaques which characterize Alzheimer’s disease. Here we analyze...
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Article
Dystrophic neurites are associated with early stage extracellular neurofibrillary tangles in the parkinsonism-dementia complex of Guam
We found tangle-associated neuritic clusters (TANCs), previously reported as being present in Alzheimer’s disease (AD), to be common in early and mild cases of the parkinsonism-dementia complex of Guam (PDC, ...
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Article
Amyloid P immunoreactivity precedes C4d deposition on extracellular neurofibrillary tangles
Extracellular neurofibrillary tangles (eNFTs) are the insoluble cytoskeletal debris left behind when neurons with intracellular neurofibrillary tangles (iNFTs) die. Reactive microglia and reactive astrocytes ...
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Article
Distinct distribution of apolipoprotein E and β-amyloid immunoreactivity in the hippocampus of Parkinson dementia complex of Guam
The distribution of apolipoprotein E (ApoE) was studied in the brain tissue of cases of the amyotrophic lateral sclerosis – parkinsonism – dementia complex of Guam, locally known as lytico bodig disease (LB),...
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Article
Relationship of amyloid β/A4 protein to the neurofibrillary tangles in Guamanian parkinsonism-dementia
The Chamorro population of the island of Guam is highly susceptible to a disease called lytico-bodig (LB), wich clinically resembles a mixture of amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD) a...
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Article
Different levels of acetylcholinesterase and choline acetyltransferase activities in C57Bl/6 and DBA/2 mice are not accompanied with different density of cortical acetylcholinesterase reactive fibers
Mice of the inbred strains C57Bl/6 and DBA/2 show strain-dependent behavioural differences which have been correlated with variations in the organization of brain cholinergic systems. The aim of our study was ...