Abstract:
:Most cases of frontotemporal lobar degeneration (FTLD) are characterized by abnormal intracellular accumulation of either tau or TDP-43 protein. However, in ~10% of cases, composed of a heterogenous collection of uncommon disorders, the molecular basis remains to be uncertain. We recently discovered that the pathological changes in several tau/TDP-43-negative FTLD subtypes are immunoreactive (ir) for the fused in sarcoma (FUS) protein. In this study, we directly compared the pattern of FUS-ir pathology in cases of atypical FTLD-U (aFTLD-U, N = 10), neuronal intermediate filament inclusion disease (NIFID, N = 5) and basophilic inclusion body disease (BIBD, N = 8), to determine whether these are discrete entities or represent a pathological continuum. All cases had FUS-ir pathology in the cerebral neocortex, hippocampus and a similar wide range of subcortical regions. Although there was significant overlap, each group showed specific differences that distinguished them from the others. Cases of aFTLD-U consistently had less pathology in subcortical regions. In addition, the neuronal inclusions in aFTLD-U usually had a uniform, round shape, whereas NIFID and BIBD were characterized by a variety of inclusion morphologies. In all cases of aFTLD-U and NIFID, vermiform neuronal intranuclear inclusions (NII) were readily identified in the hippocampus and neocortex. In contrast, only two cases of BIBD had very rare NII in a single subcortical region. These findings support aFTLD-U, NIFID and BIBD as representing closely related, but distinct entities that share a common molecular pathogenesis. Although cases with overlapping pathology may exist, we recommend retaining the terms aFTLD-U, NIFID and BIBD for specific FTLD-FUS subtypes.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Mackenzie IR,Munoz DG,Kusaka H,Yokota O,Ishihara K,Roeber S,Kretzschmar HA,Cairns NJ,Neumann Mdoi
10.1007/s00401-010-0764-0subject
Has Abstractpub_date
2011-02-01 00:00:00pages
207-18issue
2eissn
0001-6322issn
1432-0533journal_volume
121pub_type
杂志文章abstract::The blood-brain barrier (BBB) was studied in rats with electrolyte-induced demyelination (EID), an experimental model for central pontine myelinolysis. Intravenously injected peroxidase was extravasated at 3 h post hypertonic saline injection (PHS) into regions frequently involved in EID. Increased pinocytotic activit...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00310371
更新日期:1994-01-01 00:00:00
abstract::During corticogenesis, neurons adopt different migration pathways to reach their final position. The precursors of pyramidal neurons migrate radially, whereas most of the GABA-containing interneurons are generated in the ventral telencephalon and migrate tangentially into the neocortex. Then, they use a radial migrati...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0692-z
更新日期:2010-10-01 00:00:00
abstract::Long-term epidemiological studies indicate that environmental factors play a causative role in high-incidence amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) in the western Pacific. An increased risk for disease is acquired in youth and remains for life. The low concentrations of calcium and magnesi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688211
更新日期:1989-01-01 00:00:00
abstract::Virtually all phases of spinal cord injury pathogenesis, including inflammation, cell proliferation and differentiation, as well as tissue remodeling, are mediated in part by infiltrating monocyte-derived macrophages. It is now clear that these infiltrating macrophages have distinct functions from resident microglia a...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-019-01992-3
更新日期:2019-05-01 00:00:00
abstract::Keratan sulphate proteoglycan (KSPG) is a developmentally regulated barrier molecule, directing axonal growth during central nervous system (CNS) formation. The possible re-expression and functional significance of KSPG in preventing axon regeneration following spinal cord injury (SCI) is poorly understood. In the pre...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0589-6
更新日期:2002-12-01 00:00:00
abstract::In amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), spinal and lower brainstem motor neurons degenerate, but some motor neuron subtypes are spared, including oculomotor neurons (OMNs). The mechanisms responsible for this selective degeneration are largely unknown, but the molecular signatures of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02133-x
更新日期:2020-05-01 00:00:00
abstract::Localization of organ-specific brain antigens in the central nervous system of the rat has been studied by means of indirect immunofluorescence. Rabbit antiserum against homogenate of rat brain, previously absorbed with normal serum and homogenates of rat organs (kidney, liver, spleen), reacted with the water-soluble ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00703316
更新日期:1977-08-16 00:00:00
abstract::Wistar rats were given intravenous (i.v.) horseradish peroxidase (HRP) in saline in doses commonly used to study vascular permeability and the blood-brain barrier. Samples of blood were taken from conscious animals via indwelling catheters at intervals up to 6 h after the HRP injection. Plasma concentrations of adreno...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687945
更新日期:1986-01-01 00:00:00
abstract::Ubiquitin-positive Lewy neurites and Lewy bodies are found in idiopathic Parkinson's disease (PD) and diffuse Lewy body disease (DLBD). We found that, in three patients with PD and one with DLBD, microtubule-associated protein 5 (MAP5) immunostaining was consistently present in both Lewy neurites and Lewy bodies throu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050395
更新日期:1996-01-01 00:00:00
abstract::A giant-cell glioblastoma was examined by electron microscopy and by the freeze-fracture technique. The cell membranes bordering the extensive extracellular space often showed complicated undulations and peripheral vacuoles as well as occasional microvilli or filopodia. The undulations were mainly composed of plasmale...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689558
更新日期:1978-01-19 00:00:00
abstract::Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer's disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on beta-amyloid peptide (Abeta)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-009-0517-0
更新日期:2009-07-01 00:00:00
abstract::Recent studies have documented carbonyl stress involvement in the pathogenesis of sporadic amyotrophic lateral sclerosis (ALS). The aim of the present study was to assess a role for carbonyl stress in motor neuron degeneration associated with superoxide dismutase-1 (SOD1) mutant familial ALS and its transgenic mouse m...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0537-5
更新日期:2002-08-01 00:00:00
abstract::The amyloid cascade hypothesis of Alzheimer's disease (AD) is testable: it implies that interference with Abeta aggregation and plaque formation may be therapeutically useful. Abeta42 immunisation of amyloid precursor protein (APP) transgenic mice prevented plaque formation and caused removal of existing plaques. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0719-5
更新日期:2010-09-01 00:00:00
abstract::Aluminum was observed in the nucleolus, interchromatin granules, rough endoplasmic reticulum, free ribosomes, euchromatin, and the heterochromatin of the neuron. The association of aluminum with the first four r-RNA-containing cellular components and with the last two DNA-containing chromatins suggests the association...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690191
更新日期:1985-01-01 00:00:00
abstract::Mouse embryos were infected with murine cytomegalovirus (MCMV) by injecting the virus into the cerebral ventricles at the late gestation. After deliveries, offspring were fed by the mothers until 4 weeks. Cystic brain lesions, regarded as porencephaly or paraventricular cysts, were observed in about 20% of the MCMV-in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293377
更新日期:1991-01-01 00:00:00
abstract::The present study used cocultures of rat dorsal root ganglia (DRG) and peritoneal macrophages to define the role of activated complement components during demyelination. The complement cascade was activated in vitro by treatment of the cultures with natural rat serum and lipopolysaccharides. Complement activation was ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00318572
更新日期:1995-01-01 00:00:00
abstract::Neuronal accumulation of oligomeric amyloid-beta (Alphabeta) is considered the proximal cause of neuronal demise in Alzheimer disease (AD) patients. Blood-borne macrophages might reduce Abeta stress to neurons by immigration into the brain and phagocytosis of Alphabeta. We tested migration and export across a blood-br...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0481-0
更新日期:2009-02-01 00:00:00
abstract::This study was undertaken to elucidate morphological changes in the synaptic area of the Purkinje cell dendritic spines when granule cells were decreased in number. The mice were injected s.c. with 30 mg/kg b.w. of cytosine arabinoside on days 2, 3, and 4, and on days 7, 8 and 9, and were designated as group I and gro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685343
更新日期:1983-01-01 00:00:00
abstract::This study tests the effects of chronic electrostimulation on denervated/reinnervated skeletal muscle in producing an optimal restoration of size and mechanical and histochemical properties. We compared tibialis anterior muscles in four groups of rats: in unoperated control (C) and 10 weeks following nerve lesion with...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0035-2
更新日期:2006-06-01 00:00:00
abstract::Axons of the peripheral nervous system (PNS) are reduced in caliber in response to the experimental diabetic state. The cause of this reduced axonal size is disputed. Various theories include (a) axonal dwindling, (b) inhibition of growth, and (c) shrinkage due to serum hyperosmolarity. This study was designed to dire...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688637
更新日期:1985-01-01 00:00:00
abstract::A 24-year-old woman was found comatose after 2 days of cephalalgia and vomiting. An immediate diagnosis of carbon monoxyde poisoning was disclaimed when blood carbon monoxyde was found to be 1.75 ml/100. A diagnosis of acute intracranial hypertension led to trephination with ventricular punction and brain biopsy on th...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685010
更新日期:1978-08-07 00:00:00
abstract::Both protein kinases and phosphoprotein phosphatases are important components of signal transduction systems in cells. Recent studies in Alzheimer's disease (AD) have shown abnormal protein phosphorylation in the cortex suggesting an alteration in these enzymes. In the present study, an antibody against CD45 was used ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294425
更新日期:1991-01-01 00:00:00
abstract::Recent evidence suggests that brain-derived neurotrophic factor (BDNF) and its tyrosine kinase B (TrkB) receptor, in addition to promoting neuronal survival and differentiation, modulates synaptic transmission by increasing N-methyl-D-aspartic acid receptor (NMDAR) activity. Overexpression of BDNF may, then, interfere...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000296
更新日期:2001-04-01 00:00:00
abstract::A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050575
更新日期:1997-01-01 00:00:00
abstract::Recent studies have shown that Cerebrolysin can enhance synaptic function and ameliorate synaptodendritic alterations in animal models of neurodegeneration, suggesting a synaptotrophic effect. We hypothesize that Cerebrolysin might exert this effect, in part, by regulating the expression of amyloid precursor protein (...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051012
更新日期:1999-05-01 00:00:00
abstract::In almost all of the earlier reported cases of Kufs' disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination revealed an apparent poor diffusion of specific lipofuscinic deposits, the finger print profiles (FPs). We report the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050866
更新日期:1998-07-01 00:00:00
abstract::We investigated whether the brainstem is affected by the pathologic process of sporadic Creutzfeldt-Jakob disease (sCJD), with particular attention to brainstem atrophy, neuronal loss, pyramidal tract degeneration, and prion protein (PrP) deposition, in 33 patients with sCJD. Brainstem atrophy, particularly in the pon...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0981-0
更新日期:2005-06-01 00:00:00
abstract::Modern neuropathology serves a key function in the multidisciplinary management of brain tumor patients. Owing to the recent advancements in molecular neurooncology, the neuropathological assessment of brain tumors is no longer restricted to provide information on a tumor's histological type and malignancy grade, but ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0736-4
更新日期:2010-11-01 00:00:00
abstract::Structural proteins of cultured neurofibromatosis (NF) tumor and skin cells were studied with reference to control skin fibroblasts. In polyacrylamide gel electrophoresis (PAGE)/fluorography the banding patterns of the cell lysates were markedly similar. NF tumor cells, however, produced a 60 kD band with a stronger a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687332
更新日期:1984-01-01 00:00:00
abstract::Paediatric high grade glioma (pHGG) (World Health Organisation astrocytoma grades III and IV) remains poor prognosis tumours, with a median survival of only 15 months following diagnosis. Current investigation of anti-angiogenic strategies has focused on adult glioblastoma multiforme (GBM) with phase III trials target...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-0952-1
更新日期:2012-07-01 00:00:00