Abstract:
:The transmissible spongiform encephalopathies (TSEs) or prion diseases of animals are characterised by CNS spongiform change, gliosis and the accumulation of disease-associated forms of prion protein (PrP(d)). Particularly in ruminant prion diseases, a wide range of morphological types of PrP(d) depositions are found in association with neurons and glia. When light microscopic patterns of PrP(d) accumulations are correlated with sub-cellular structure, intracellular PrP(d) co-localises with lysosomes while non-intracellular PrP(d) accumulation co-localises with cell membranes and the extracellular space. Intracellular lysosomal PrP(d) is N-terminally truncated, but the site at which the PrP(d) molecule is cleaved depends on strain and cell type. Different PrP(d) cleavage sites are found for different cells infected with the same agent indicating that not all PrP(d) conformers code for different prion strains. Non-intracellular PrP(d) is full-length and is mainly found on plasma-lemmas of neuronal perikarya and dendrites and glia where it may be associated with scrapie-specific membrane pathology. These membrane changes appear to involve a redirection of the predominant axonal trafficking of normal cellular PrP and an altered endocytosis of PrP(d). PrP(d) is poorly excised from membranes, probably due to increased stabilisation on the membrane of PrP(d) complexed with other membrane ligands. PrP(d) on plasma-lemmas may also be transferred to other cells or released to the extracellular space. It is widely assumed that PrP(d) accumulations cause neurodegenerative changes that lead to clinical disease. However, when different animal prion diseases are considered, neurological deficits do not correlate well with any morphological type of PrP(d) accumulation or perturbation of PrP(d) trafficking. Non-PrP(d)-associated neurodegenerative changes in TSEs include vacuolation, tubulovesicular bodies and terminal axonal degeneration. The last of these correlates well with early neurological disease in mice, but such changes are absent from large animal prion disease. Thus, the proximate cause of clinical disease in animal prion disease is uncertain, but may not involve PrP(d).
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Jeffrey M,McGovern G,Sisó S,González Ldoi
10.1007/s00401-010-0700-3subject
Has Abstractpub_date
2011-01-01 00:00:00pages
113-34issue
1eissn
0001-6322issn
1432-0533journal_volume
121pub_type
杂志文章,评审abstract::Mice develop a fatal encephalomyelitis after infection with the Trinidad donkey strain of Venezuelan equine encephalitis (VEE) virus. Adult mice were inoculated intraperitoneally with VEE virus and the brains were examined at different time points. Morphological changes were assessed by histological staining. VEE viru...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050626
更新日期:1997-04-01 00:00:00
abstract::Thirty-one squirrel monkey fetuses were exposed to lead acetate given to the mothers perorally during the last three-fourths or two-thirds of pregnancy. The mean maternal blood lead concentration of the group was 37 micrograms/100 ml and the individual means ranged from 22-82 micrograms/100 ml. Examination of the cent...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687421
更新日期:1988-01-01 00:00:00
abstract::The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688176
更新日期:1989-01-01 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::Granule-containing vacuoles in the cytoplasm of hippocampal neurons are a neuropathological feature of Alzheimer's disease. Granulovacuolar degeneration (GVD) is not disease-specific and can be observed in other neurodegenerative disorders and even in the brains of non-demented elderly people. However, several studies...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-016-1562-0
更新日期:2016-09-01 00:00:00
abstract::A study was undertaken to determine the pathological significance of previously unrecognized intracytoplasmic eosinophilic inclusions (IEIs) in ependymoma. The study group consisted of 58 ependymomas, all of which were pathologically characterized and graded according to the 1993 WHO classification. Electron microscop...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007427
更新日期:2000-02-01 00:00:00
abstract::Numbers of dystrophic neurites, seen with the electron microscope, in CA1 of the hippocampus of either C3H mice infected with 22C or 79A strains of scrapie, or LM mice infected with strain ME7 were greater than in age-matched control mice. Vacuolation, seen by light microscopy in CA1 of the hippocampus of mice infecte...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688263
更新日期:1987-01-01 00:00:00
abstract::Data on the dural invasiveness of pituitary adenomas have been correlated to the expression of matrix metalloproteinases (e.g. MMP-9). Serine proteases have not yet been investigated in human pituitary adenomas. In this study, paraffin-embedded material from 84 human pituitary adenomas (acromegaly n=18, Cushing's dise...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0747-5
更新日期:2003-11-01 00:00:00
abstract::A newly developed in vitro labeling method with bromodeoxyuridine (BrdU) identifies S phase cells in situ in freshly obtained surgical tissue of human brain tumors which is subsequently fixed and embedded in paraffin for BrdU immunovisualization. For the first time, the BrdU labeling index (LI) is successfully compare...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687579
更新日期:1989-01-01 00:00:00
abstract::Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to D. The strength of this histopathologic classification lies in the association between FTLD-TDP subtypes and various clinical and genetic f...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-017-1679-9
更新日期:2017-07-01 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::This report concerns an immunocytochemical and ultrastructural study of the motor cortices of 11 patients with amyotrophic lateral sclerosis (ALS). Specimens from 12 normal individuals served as controls. Antibodies against phosphorylated neurofilament (PNF; 200 kDa), ubiquitin, glial fibrillary acidic protein (GFAP) ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293318
更新日期:1994-01-01 00:00:00
abstract::Using a fluorescent antibody technique, sections of the brains of patients with senile dementia or Alzheimer's disease containing senile plaques were treated with rabbit antihuman immunoglobulins labelled with FITC (fluorescein isothiocyanate, BBL). The senile plaques and the cerebral amyloid angiopathy (drusige Entar...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689569
更新日期:1975-08-11 00:00:00
abstract::We investigated the distribution of prion protein (PrP) in 14 German patients with sporadic Creutzfeldt-Jakob disease (CJD) and compared it with that observed in Japanese patients. Immunohistochemical study revealed diffuse gray matter stainings including synaptic structures in all cases. In addition, 4 patients showe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294174
更新日期:1994-01-01 00:00:00
abstract::The occurrence of oligodendrocytes within astrocytes ("emperipolesis") has been described in demyelinating lesions in cases of multiple sclerosis and also in other non-demyelinating disorders. We found that this finding was common in the cerebral white matter of patients with Creutzfeldt-Jakob disease (CJD). Eight con...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0880-9
更新日期:2004-09-01 00:00:00
abstract::A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690395
更新日期:1984-01-01 00:00:00
abstract::We studied a 27-year-old woman who died after a 6-year history of progressive dementia, dystonia, ataxia, apraxia, spasticity, choreoathetosis, visual and auditory hallucinations, and optic atrophy. Magnetic resonance imaging showed decreased intensity in the globus pallidus, substantia nigra, and dentate nuclei in T2...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000224
更新日期:2000-11-01 00:00:00
abstract::Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrP(Sc), and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenot...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0585-1
更新日期:2009-11-01 00:00:00
abstract::A 9-year-old male patient developed a germ cell tumor in the right basal ganglia which secreted beta-human chorionic gonadotropin (beta-HCG) and caused precocious puberty. Histology and immunohistochemical staining for placental alkaline phosphatase (PLAP), alpha-fetoprotein (alpha-FP), and beta-HCG showed a mixed pop...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687797
更新日期:1988-01-01 00:00:00
abstract::The left and right recurrent laryngeal nerves and peroneal nerves from two groups of foals, one less than 1 month of age and the other 6 months of age, were examined by light and electron microscopy. While there was no evidence of fiber loss on light microscopy, occasional onion bulbs, regenerating clusters and swolle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227825
更新日期:1992-01-01 00:00:00
abstract::Nodding syndrome is an epidemic neurologic disorder of unknown cause that affects children in the subsistence-farming communities of East Africa. We report the neuropathologic findings in five fatal cases (13-18 years of age at death) of nodding syndrome from the Acholi people in northern Uganda. Neuropathologic exami...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1909-9
更新日期:2018-11-01 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::Cyclooxygenases (COX, prostaglandin endoperoxide synthases, PGG/H synthases) are potent mediators of inflammation. While COX-1 is constitutively expressed in a wide range of tissues, COX-2 is cytokine inducible. Although COX-1 expression is observed in normal tissue, enhanced COX-2 expression has been attributed a key...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051075
更新日期:1999-09-01 00:00:00
abstract::The colocalization of beta amyloid protein with the enzymes acetyl- and butyrylcholinesterase was assessed using immunocytochemistry for beta amyloid protein and a sensitive histochemical technique for cholinesterases. In non-demented aged and Alzheimer's disease brains, double-stained sections for cholinesterases and...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334445
更新日期:1993-01-01 00:00:00
abstract::Upon brain reperfusion following ischemia, there is widespread inhibition of neuronal protein synthesis that is due to phosphorylation of eukaryotic initiation factor 2alpha (eIF2alpha), which persists in selectively vulnerable neurons (SVNs) destined to die. Other investigators have shown that expression of mutant eI...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0693-2
更新日期:2003-07-01 00:00:00
abstract::The calcium-activated protease calpain cleaves a variety of biologically important proteins and serves, therefore, as a key regulator of many cellular functions. Activation of both main isoforms, calpain 1 and calpain 2, was demonstrated previously in Alzheimer's disease. In this report, antibodies specifically recogn...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0528-6
更新日期:2002-07-01 00:00:00
abstract::The identification of the alpha-synuclein gene on chromosome 4q as a locus for familial Lewy-body parkinsonism and of alpha-synuclein as a component of Lewy bodies has heralded a new era in the study of Parkinson's disease. We have identified a large family with Lewy body parkinsonism linked to a novel locus on chromo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051177
更新日期:2000-06-01 00:00:00
abstract::Gelatinase A is an enzyme capable of cleaving soluble beta-amyloid protein (beta AP), and may function as an alpha-secretase to produce secretory forms of amyloid precursor protein. We examined gelatinase A immunoreactivity in the brains and posterior roots of neurologically normal, lacunar stroke, Alzheimer disease (...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309334
更新日期:1995-01-01 00:00:00
abstract::In this report, we describe the clinical, topographical and immunohistochemical characteristics of neurofilament (NF) inclusion formation induced by the intracisternal inoculation of young adult New Zealand white rabbits at 28-day intervals with 100 micrograms AlCl3 over the course of 267 days. The ability to recover ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294460
更新日期:1995-01-01 00:00:00
abstract::Microglia are long-living resident immune cells of the brain, which secure a stable chemical and physical microenvironment necessary for the proper functioning of the central nervous system (CNS). These highly dynamic cells continuously scan their environment for pathogens and possess the ability to react to damage-in...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-015-1524-y
更新日期:2016-03-01 00:00:00