Abstract:
:Clinical and morphological findings in an 8-year-old Jewish girl with spongy leukodystrophy are presented. Ultrastructural changes indicated that this may be a form of Canavan's disease with some atypical features. Biochemical measurements indicated that synaptosomal membranes prepared from the striatum but not from the cerebral cortex were deficient in protein kinase activity based on incorporation of 32P into proteins. Both areas contained the same spectrum of proteins. Cyclic AMP (cAMP) measurements indicated no significant differences between brain areas, or when compared to tissue obtained from a stillborn and a 5-year-old child.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Boehme DH,Marks Ndoi
10.1007/BF00691321subject
Has Abstractpub_date
1981-01-01 00:00:00pages
221-5issue
3eissn
0001-6322issn
1432-0533journal_volume
55pub_type
杂志文章abstract::The caudate nucleus and putamen belong to the selectively vulnerable brain regions which incur neuronal damage in clinical and experimental settings of both hypoglycemia and ischemia. We have previously documented the density and distribution of the hypoglycemic damage in rat caudoputamen, but the evolution of the inj...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688122
更新日期:1985-01-01 00:00:00
abstract::Alpha-synuclein is known to play an important role in several neurodegenerative diseases. Moreover, it is expressed in central nervous system neuronal tumors, and another member of the synuclein family, gamma-synuclein, is overexpressed in breast and ovarian carcinomas. However, the expression of alpha-synuclein has n...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100443
更新日期:2002-02-01 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
abstract::An autopsy case of unclassifiable presenil dementia is reported. The outstanding pathological findings were as follows; 1. presence of senile plaques, neurofibrillary changes, Pick bodies, Hirano bodies, granulovacuolar degeneration of neurons, etc. 2. numerous Lewy bodies in the brain stem and diencephalon, 3. peculi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685366
更新日期:1976-11-15 00:00:00
abstract::Amplification of the epidermal growth factor receptor (EGFR) gene is found in about 40% of glioblastomas (GBMs) but is rarely detected in GBM cell lines. We confirmed that the exceptional SKMG-3 GBM cell line retained amplified EGFR genes in vitro, and found that these sequences were concentrated on extra-chromosomal ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000332
更新日期:2001-06-01 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::Disturbance of intracellular trafficking plays a major role in several neurodegenerative disorders including Alzheimer or Parkinson's disease. The Chediak-Higashi syndrome (CHS), a life-threatening autosomal recessive disease with frequent mutations in the LYST gene, and its animal model, the beige mouse, are both cha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0092-6
更新日期:2006-09-01 00:00:00
abstract::Monosodium-L-glutamate given subcutaneously to pregnant rats caused acute necrosis of the acetylcholinesterase-positive neurons in the area postrema. The same effect has been observed in the area postrema of fetal rats. The process of neuronal cell death and the elimination of debris by microglia cells proved to be si...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686787
更新日期:1987-01-01 00:00:00
abstract::This paper examines the neuropathology of oxygen-glucose deprivation uncomplicated by stagnant conditions. Rabbit vagus nerves were pulled into a multi-compartment perfusion chamber, stimulated five times per second and deprived of energy by substituting nitrogen and deoxyglucose for oxygen and glucose in the Locke's ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685378
更新日期:1982-01-01 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::This report presents the ultrastructural nerve study of a patient with sensorimotor neuropathy by a shoe glue containing an association of n-hexane and methylethylketone. Giant axons, distended by microfilamentous proliferation typical of such cases of neuropathy, were found in significant amounts. An unusual histolog...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690990
更新日期:1981-01-01 00:00:00
abstract::Alzheimer's disease (AD) is a major cause of dementia. Characteristic neuropathological features of AD include neurofibrillary tangles, senile plaques, amyloid angiopathy and microvascular atrophy. The ultra-structure of the microvascular atrophy in AD and its pathogenetic significance have not been defined. This repo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050386
更新日期:1996-01-01 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::Circumscribed pink areas 0.3-1.0 cm in diameter seen in deeper parts of otherwise well-fixed brains are usually ascribed to inadequate fixation. Twenty-three patients with pink areas in their fixed brains had evidence of inflammation in at least one organ. Blood vessels in the pink areas contained bacteria. Postmortem...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687020
更新日期:1985-01-01 00:00:00
abstract::Prions cause scrapie and Creutzfeldt-Jakob disease (CJD); these infectious pathogens are composed largely, if not entirely, of protein molecules. No prion-specific polynucleotide has been identified. Purified preparations of scrapie prions contain high titers (greater than or equal to 10(9.5) ID50/ml), one protein (Pr...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/BF00687261
更新日期:1987-01-01 00:00:00
abstract::The mechanism of antibody-mediated central nervous system (CNS) demyelination in vivo was studied using rabbit eyes. Injection of anti-galactocerebroside (Gal C) antiserum alone into the normal rabbit vitreous body induced demyelination in the epiretinal myelinated fibers. This activity of the antiserum disappeared af...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687890
更新日期:1989-01-01 00:00:00
abstract::The selective toxicity of silica dust for macrophages has been used to assess the role of these cells in experimental allergic neuritis (EAN). Inbred Lewis rats were inoculated with bovine dorsal roots in Freund's complete adjuvant (day 0). In two experiments, animals received 200 mg of silica dust in 1 cm3 of saline ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687614
更新日期:1984-01-01 00:00:00
abstract::The accumulation of misfolded α-synuclein (aSyn) and neuron loss define several neurodegenerative disorders including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, the precise relationship between pathology and neurotoxicity and why these processes disproportionately affect certain neuron subp...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1829-8
更新日期:2018-06-01 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::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::The authors present the case of a hypertensive patient with a thrombosis of a saccular microaneurysm (SMA) and underlying cerebral (pontine) lacunae. This SMA lay at the junction between the main vessel and two small feeding arteries penetrating into the necrotic territory. Microhemorrhages were observed in and outsid...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688313
更新日期:1986-01-01 00:00:00
abstract::Spinal cord of the rat was investigated immunohistochemically to detect signs of extravasation of fibronectin in animals in which the cord was subjected to different degrees of compression trauma. Immunohistochemistry was performed after survival periods of 4 and 24 h and parallel sections were incubated for albumin i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227738
更新日期:1992-01-01 00:00:00
abstract::An extensive laminar spongiosis was found in the outer part of the dentate gyrus in an 84-year-old patient. An old cavitary infarct in the parahippocampal gyrus disconnected the dentate gyrus from the entorhinal area. This finding prompted us to seek laminar spongiosis in Alzheimer's disease, where the neuronal loss i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050818
更新日期:1998-04-01 00:00:00
abstract::Continuous intrathecal infusion of N-methyl-D-aspartate (NMDA) at the level of the lumbar enlargement of the spinal cord in middle-aged rats produced dose-dependent toxicity of spinal cord neuronal systems. Toxicity was enhanced by coadministration of glycine, but was significantly reduced when NMDA was co-administere...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00662631
更新日期:1990-01-01 00:00:00
abstract::The neurone-centred view of the past disregarded or downplayed the role of astroglia as a primary component in the pathogenesis of neurological diseases. As this concept is changing, so is also the perceived role of astrocytes in the healthy and diseased brain and spinal cord. We have started to unravel the different ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-015-1513-1
更新日期:2016-03-01 00:00:00
abstract::A macrosomic male infant with multiple malformations survived for 4 days. His external dysplasias comprised macrocephalus, cheilopalatoschisis, auricular anomalies, and unilateral hexadactyl; his internal dysplasias included cysts of kidneys and pancreas, and a patent foramen ovale. The child had frequent generalized ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690449
更新日期:1980-01-01 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::Aging and neurodegeneration are often accompanied by a functionally impaired ubiquitin-proteasome system (UPS). In tauopathies and polyglutamine diseases, a mutant form of ubiquitin B (UBB(+1)) accumulates in disease-specific aggregates. UBB(+1) mRNA is generated at low levels in vivo during transcription from the ubi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1003-7
更新日期:2012-08-01 00:00:00
abstract::Aberrant peripheral nerve fiber bundles, i.e., masses of peripheral nerve fibers without perineural sheath cells, are found in the perivascular spaces of some spinal cords. The 514 spinal cords examined for these structures were separated into two groups; 507 spinal cords without definite necrotic lesions (group 1) an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308951
更新日期:1989-01-01 00:00:00
abstract::A case of Creutzfeldt-Jakob disease (CJD) with a rare mutation of the prion protein (PrP) gene (PRNP) at codon 208 (R208H) is described. By comparison with two preceding reports, the case described here displayed two distinct biochemical and neuropathological features. Western blot analysis of brain homogenates showed...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0978-0
更新日期:2005-04-01 00:00:00