The unfolded protein response in neurodegenerative diseases: a neuropathological perspective.

abstract：:Adult white rats were injected daily for 1 month with aluminum chloride and killed. Aluminum was identified, quantitated, and localized in the brains of these rats. The only detectable anomalies were crystals of aluminum and phosphorus in lysosomes and lipofuscin granules in the cytoplasm of neurons. ...

journal_title：Acta neuropathologica

authors： Galle P,Berry JP,Duckett S

更新日期：1980-01-01 00:00:00

abstract：:Round granulated body (RGB) and eosinophilic hyaline droplets (EHDs) have been described as cytoplasmic inclusions of certain astrocytic tumors. In the previous literature, however, these inclusions have been described using various terms or regarded as nosologically the same entity. Light microscopically, RGB appeare...

journal_title：Acta neuropathologica

authors： Hitotsumatsu T,Iwaki T,Fukui M,Tateishi J

更新日期：1994-01-01 00:00:00

abstract：:α-Synuclein (αSyn) histopathology defines several neurodegenerative disorders, including Parkinson's disease, Lewy body dementia, and Alzheimer's disease (AD). However, the functional link between soluble αSyn and disease etiology remains elusive, especially in AD. We, therefore, genetically targeted αSyn in APP trans...

journal_title：Acta neuropathologica

authors： Khan SS,LaCroix M,Boyle G,Sherman MA,Brown JL,Amar F,Aldaco J,Lee MK,Bloom GS,Lesné SE

更新日期：2018-10-01 00:00:00

abstract：:Neuroaxonal spheroids became evident microscopically after the autopsy of a 45-year-old woman with pigmentation of the globus pallidus suggesting Hallervorden-Spatz disease. In our opinion the fine floccular pigment seen electron-microscopically in many of the axonal spheroids is melanin, an end product of catecholami...

journal_title：Acta neuropathologica

authors： Hartmann HA,White SK,Levine RL

更新日期：1983-01-01 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

authors： Page AB,Owen CR,Kumar R,Miller JM,Rafols JA,White BC,DeGracia DJ,Krause GS

更新日期：2003-07-01 00:00:00

abstract：:Altered levels of cerebrospinal fluid (CSF) peptides related to Alzheimer's disease (AD) are associated with pathologic AD diagnosis, although cognitively normal subjects can also have abnormal levels of these AD biomarkers. To identify novel CSF biomarkers that distinguish pathologically confirmed AD from cognitively...

journal_title：Acta neuropathologica

authors： Hu WT,Chen-Plotkin A,Arnold SE,Grossman M,Clark CM,Shaw LM,Pickering E,Kuhn M,Chen Y,McCluskey L,Elman L,Karlawish J,Hurtig HI,Siderowf A,Lee VM,Soares H,Trojanowski JQ

更新日期：2010-06-01 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

authors： Roeber S,Krebs B,Neumann M,Windl O,Zerr I,Grasbon-Frodl EM,Kretzschmar HA

更新日期：2005-04-01 00:00:00

abstract：:Pediatric glioblastomas (GBM) including diffuse intrinsic pontine gliomas (DIPG) are devastating brain tumors with no effective therapy. Here, we investigated clinical and biological impacts of histone H3.3 mutations. Forty-two DIPGs were tested for H3.3 mutations. Wild-type versus mutated (K27M-H3.3) subgroups were c...

journal_title：Acta neuropathologica

authors： Khuong-Quang DA,Buczkowicz P,Rakopoulos P,Liu XY,Fontebasso AM,Bouffet E,Bartels U,Albrecht S,Schwartzentruber J,Letourneau L,Bourgey M,Bourque G,Montpetit A,Bourret G,Lepage P,Fleming A,Lichter P,Kool M,von Deimling

更新日期：2012-09-01 00:00:00

abstract：:Hydrocephalic neonates were observed in a small breeding colony of rats. Normal rats from this colony were obtained and brother-sister mated for seven generations. The overall prevalence of hydrocephalics was approximately 23%; however, in one subline, the prevalence approached 50%. Breeding data suggested the trait t...

journal_title：Acta neuropathologica

authors： Kohn DF,Chinookoswong N,Chou SM

更新日期：1981-01-01 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

authors： Krautstrunk M,Scholtes F,Martin D,Schoenen J,Schmitt AB,Plate D,Nacimiento W,Noth J,Brook GA

更新日期：2002-12-01 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

authors： Kosaka K,Oyanagi S,Matsushita M,Hori A

更新日期：1976-11-15 00:00:00

abstract：:A limited cortical resection including the rolandic fissure and the pre- and postcentral cortical regions was carried out in a patient suffering from epilepsia partialis continua resistant to antiepileptic drugs. The histological examination revealed several foci of very large neurons distributed with no laminar organ...

journal_title：Acta neuropathologica

authors： Ferrer I,Pineda M,Tallada M,Oliver B,Russi A,Oller L,Noboa R,Zújar MJ,Alcántara S

更新日期：1992-01-01 00:00:00

abstract：:A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer...

journal_title：Acta neuropathologica

authors： Miyakawa T,Murayama E

更新日期：1976-01-01 00:00:00

abstract：:Bundles of paired helical filaments (PHF) accumulate in the pyramidal neurons that degenerate during Alzheimer's disease. This neurofibrillary degeneration is highly correlated with clinical signs of dementia. During the degenerating process, Tau proteins, which are the major antigenic components of PHF, are abnormall...

journal_title：Acta neuropathologica

authors： Delacourte A,Flament S,Dibe EM,Hublau P,Sablonnière B,Hémon B,Shérrer V,Défossez A

更新日期：1990-01-01 00:00:00

abstract：:In comparison to the levels in age and gender-matched controls, reduced levels of pathological amyloid-β protein in cerebrospinal fluid routinely precede the onset of Alzheimer's disease-related symptoms by several years, whereas elevated soluble abnormal tau fractions (phosphorylated tau, total tau protein) in cerebr...

journal_title：Acta neuropathologica

authors： Braak H,Zetterberg H,Del Tredici K,Blennow K

更新日期：2013-11-01 00:00:00

abstract：:Glucosylceramide lipidosis results from a defective lysosomal degradation of this glycolipid. Lipid degradation is controlled by two components, the enzyme beta-glucocerebrosidase and a sphingolipid activator protein. While most Gaucher cases are due to mutations within the gene that codes for the lysosomal enzyme, on...

journal_title：Acta neuropathologica

authors： Pàmpols T,Pineda M,Girós ML,Ferrer I,Cusi V,Chabás A,Sanmarti FX,Vanier MT,Christomanou H

更新日期：1999-01-01 00:00:00

abstract：:Pathways for drainage of interstitial fluid and cerebrospinal fluid from the rat brain were investigated by the injection of 2-5 microliters Indian ink into cerebral white and grey matter and into the subarachnoid space over the vertex of the left frontal lobe. Animals were killed by formalin or glutaraldehyde perfusi...

journal_title：Acta neuropathologica

authors： Zhang ET,Richards HK,Kida S,Weller RO

更新日期：1992-01-01 00:00:00

abstract：:In 54 cats experimental brain tumors were produced by xenotransplantation of the blastomatous glial cell clone RG2 into the internal capsule of the left hemisphere. Fifteen of these animals were treated with dexamethasone for 1 week and four animals for 2 h. The occurrence of glial fibrillary acidic (GFA) protein in t...

journal_title：Acta neuropathologica

authors： Szymas J,Hossmann KA

更新日期：1984-01-01 00:00:00

abstract：:Two young zebra siblings from consecutive pregnancies suffered from neurological disease, characterized by ventral deviation of the neck and tetraparesis which progressed to tetraplegia within a few weeks. On histological examination widespread neuronal degeneration was observed in the ventral horns of the spinal cord...

journal_title：Acta neuropathologica

authors： Higgins RJ,Vandevelde M,Hoff EJ,Jagar JE,Cork LC,Silberman MS

更新日期：1977-01-31 00:00:00

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 pathologi...

journal_title：Acta neuropathologica

authors： Mackenzie IR,Munoz DG,Kusaka H,Yokota O,Ishihara K,Roeber S,Kretzschmar HA,Cairns NJ,Neumann M

更新日期：2011-02-01 00:00:00

abstract：:The ultrastructural features of SOD1-positive aggregates were determined to clarify whether these aggregates are associated with the pathogenesis of SOD1 mutant mice. We examined the spinal cord of transgenic mice expressing a G93A mutant human SOD1 gene with fewer copies (gene copy 10). At the early presymptomatic st...

journal_title：Acta neuropathologica

authors： Sasaki S,Warita H,Murakami T,Shibata N,Komori T,Abe K,Kobayashi M,Iwata M

更新日期：2005-03-01 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

authors： Griot C,Bürge T,Vandevelde M,Peterhans E

更新日期：1989-01-01 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

authors： Aguzzi A,Hedinger CE,Kleihues P,Yaşargil MG

更新日期：1988-01-01 00:00:00

abstract：:Spinal cord-notochord relationship was analyzed histologically and immunohistochemically in normal human conceptuses between the 4-8 developmental weeks and in a 8-week embryo with double spinal cord. In the early 4-week embryo, the gradual closure of the neural tube along the cranio-caudal body axis was paralleled by...

journal_title：Acta neuropathologica

authors： Saraga-Babić M,Stefanović V,Wartiovaara J,Lehtonen E

更新日期：1993-01-01 00:00:00

abstract：:Changes in morphology, behavior of the blood-brain barrier (BBB), regional cerebral blood flow (rCBF), and local cerebral glucose utilization (LCGU) were assessed and correlated in Mongolian gerbils following 5 min cerebral ischemia, produced by bilateral clamping of the common carotid arteries. The morphological chan...

journal_title：Acta neuropathologica

authors： Suzuki R,Yamaguchi T,Kirino T,Orzi F,Klatzo I

更新日期：1983-01-01 00:00:00

abstract：:Glycerol (an atoxic alcohol) and phenol (a toxic monohydroxybenzene) are currently used as neurolytic blocking agents to relieve pain or spasticity. In the present study we compared the endoneurial response of anhydrous glycerol and 7% phenol-aqua after intraneural injection into rat sciatic nerve, using electron micr...

journal_title：Acta neuropathologica

authors： Westerlund T,Vuorinen V,Röyttä M

更新日期：2001-07-01 00:00:00

abstract：:3-Nitropropionic acid (NPA), a toxin which irreversibly inhibits the Krebs cycle enzyme succinate dehydrogenase, causes severe neurologic disease and a specific pattern of morphologic brain damage when given subcutaneously to rats. To determine whether hypotension or hypoxemia were necessary for development of morphol...

journal_title：Acta neuropathologica

authors： Hamilton BF,Gould DH

更新日期：1987-01-01 00:00:00

abstract：:The cholinergic basal forebrain is divided into four subregions (Ch1-4), and cholinergic neuronal loss in the nucleus basalis of Meynert (Ch4) has been correlated with cognitive impairments in both Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). However, the Ch1-2 regions, which provide the major choline...

journal_title：Acta neuropathologica

authors： Fujishiro H,Umegaki H,Isojima D,Akatsu H,Iguchi A,Kosaka K

更新日期：2006-02-01 00:00:00

abstract：:Activation of the MAPK signaling pathway has been shown to be a unifying molecular feature in pilocytic astrocytoma (PA). Genetically, tandem duplications at chromosome 7q34 resulting in KIAA1549-BRAF fusion genes constitute the most common mechanism identified to date. To elucidate alternative mechanisms of aberrant ...

journal_title：Acta neuropathologica

authors： Cin H,Meyer C,Herr R,Janzarik WG,Lambert S,Jones DT,Jacob K,Benner A,Witt H,Remke M,Bender S,Falkenstein F,Van Anh TN,Olbrich H,von Deimling A,Pekrun A,Kulozik AE,Gnekow A,Scheurlen W,Witt O,Omran H,Jabado N,C

更新日期：2011-06-01 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

authors： Tsutsui Y,Kashiwai A,Kawamura N,Kadota C,Nagahama M

更新日期：1991-01-01 00:00:00