FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy.

abstract：:The neuropathological staging model of Alzheimer's disease proposed by Braak and Braak [Acta Neuropathol (1991) 82:259] requires that the evolution of neurofibrillary pathology follows a predictable pattern that can be ordered in a regular regional hierarchy. We have operationalized the neuropathological staging syste...

journal_title：Acta neuropathologica

authors： Gertz HJ,Xuereb J,Huppert F,Brayne C,McGee MA,Paykel E,Harrington C,Mukaetova-Ladinska E,Arendt T,Wischik CM

更新日期：1998-02-01 00:00:00

abstract：:In 2011, genome-wide association studies implicated a polymorphism near CD33 as a genetic risk factor for Alzheimer's disease. This finding sparked interest in this member of the sialic acid-binding immunoglobulin-type lectin family which is linked to innate immunity. Subsequent studies found that CD33 is expressed in...

journal_title：Acta neuropathologica

authors： Estus S,Shaw BC,Devanney N,Katsumata Y,Press EE,Fardo DW

更新日期：2019-08-01 00:00:00

abstract：:In a large series of 116 cortical resections for treatment of medically intractable epilepsy, 10 glial hamartomas and 11 neuronoglial malformative lesions were found. Glial hamartomas were astrocytic in 3 cases, oligodendrocytic in 6 and mixed oligoastrocytic in 1. Neuronoglial lesions corresponded to "focal cortical ...

journal_title：Acta neuropathologica

authors： Vital A,Marchal C,Loiseau H,Rougier A,Pedespan JM,Rivel J,Vital C

更新日期：1994-01-01 00:00:00

abstract：:The number and nucleolar volume of nerve cells within the nucleus basalis of Meynert, locus caeruleus, substantia nigra and dorsal raphe were examined in five patients with Huntington's chorea. No significant changes in nerve cell number were noted in any area in any patient and, although nucleolar volume was reduced ...

journal_title：Acta neuropathologica

authors： Mann DM

更新日期：1989-01-01 00:00:00

abstract：:Double immunofluorescence for paired helical filament (PHF)-tau (AT8) and ubiquitin, enhanced by catalyzed reporter deposition amplification, was combined with thiazin red (TR), a fluorochrome, which has an affinity to fibrillary structures such as neurofibrillary tangles (NFTs). After recording these triple-fluoresce...

journal_title：Acta neuropathologica

authors： Uchihara T,Nakamura A,Yamazaki M,Mori O

更新日期：2001-06-01 00:00:00

abstract：:Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor ...

journal_title：Acta neuropathologica

authors： Uchihara T,Ohashi K,Kitagawa M,Kurata M,Nakamura A,Hirokawa K,Kasuga T,Kobayashi T

更新日期：2010-01-01 00:00:00

abstract：:It has been suggested that delayed development in either the peripheral or central nervous system could underlie the sudden infant death syndrome (SIDS). We studied the phrenic nerve in an attempt to find if maturation in this nerve was delayed in SIDS, and to see if fiber size differences could explain the paucity of...

journal_title：Acta neuropathologica

authors： Pamphlett R,Murray N,Louda C

更新日期：1996-01-01 00:00:00

abstract：:A 1-year-old male chinchilla with a 2-week history of conjunctivitis suffered subsequently from neurological signs comprising seizures, disorientation, recumbency and apathy. After 3 weeks of progressive central nervous disease the animal was killed in view of the poor prognosis. A non-suppurative meningitis and polio...

journal_title：Acta neuropathologica

authors： Wohlsein P,Thiele A,Fehr M,Haas L,Henneicke K,Petzold DR,Baumgärtner W

更新日期：2002-12-01 00:00:00

abstract：:A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...

journal_title：Acta neuropathologica

authors： Kosaka K,Mehraein P

更新日期：1978-12-15 00:00:00

abstract：:A significant reduction in the myelinated nerve fiber population was observed during quantitative electron-microscopic examination of peripheral nerves in chronic alloxan diabetic rats. Dystrophic axonal abnormalities and regenerating fibers were more numerous in diabetics than age-matched controls. Schwann cells show...

journal_title：Acta neuropathologica

authors： Powell HC,Myers RR

更新日期：1984-01-01 00:00:00

abstract：:The cerebrovascular lesions of severe chronic hypertension were studied by light microscopy in perfusion-fixed, subserially sectioned brains from stroke-prone spontaneously hypertensive rats (SHRSP). The leakage and spread of plasma proteins were visualized by immunohistochemical detection of extravasated fibrinogen a...

journal_title：Acta neuropathologica

authors： Fredriksson K,Auer RN,Kalimo H,Nordborg C,Olsson Y,Johansson BB

更新日期：1985-01-01 00:00:00

abstract：:Seven patients aged 29 to 76 years with various clinical subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) were investigated. Sural nerve biopsies were performed between 7 months and 19 years after onset of disease. Quantitative electron microscopy revealed involvement of primary unmyelinated fibers...

journal_title：Acta neuropathologica

authors： Gibbels E,Kentenich M

更新日期：1990-01-01 00:00:00

abstract：:A quantitative technique involving serial sectioning and semiautomatic morphometric analysis was used to assess the severity of the reduction in size of the major brain structures in cerebral hemispheres of children congenitally infected with HIV-1. Cerebral hemispheres from 12 children (18-48 months of age) who died ...

journal_title：Acta neuropathologica

authors： Kozlowski PB,Brudkowska J,Kraszpulski M,Sersen EA,Wrzolek MA,Anzil AP,Rao C,Wisniewski HM

更新日期：1997-02-01 00:00:00

abstract：:We present the neuropathological findings in a female patient with clinically definite multiple sclerosis (MS), who at autopsy had multifocal amyloid deposits in the white matter of the brain without other signs of amyloidosis. The patient had relapsing/remitting MS between the ages of 26 and 45, and during her last 1...

journal_title：Acta neuropathologica

authors： Nennesmo I,Bogdanovic N,Petrén AL,Fredrikson S

更新日期：1997-02-01 00:00:00

abstract：:Fifteen cases of mitochondrial myopathy, three cases of hereditary motor and sensory neuropathy (HMSN) VI, and 280 cases of neuropathies of different etiologies were examined by electron microscopy for the presence of mitochondrial abnormalities in the sural nerve. Altered mitochondrial were found in most cases of mit...

journal_title：Acta neuropathologica

authors： Schröder JM,Sommer C

更新日期：1991-01-01 00:00:00

abstract：:We have studied microtubule-associated protein 2 (MAP2) expression in anterior horn neurons in the cervical and lumbar spinal cords of 19 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS) using immunohistochemistry. Specimens from 7 patients without neurological disease served as controls. MAP2 express...

journal_title：Acta neuropathologica

authors： Kikuchi H,Doh-ura K,Kawashima T,Kira J,Iwaki T

更新日期：1999-01-01 00:00:00

abstract：:We report a sporadic tauopathy of 6-year duration in a 76-year-old woman. Her initial symptoms were asymmetrical parkinsonism and muscle weakness, with apraxia appearing 2 years later. The brain showed frontal and temporal cerebral atrophy; severe neuronal loss and gliosis were observed in the precentral cortex (loss ...

journal_title：Acta neuropathologica

authors： Piao YS,Tan CF,Iwanaga K,Kakita A,Takano H,Nishizawa M,Lashley T,Revesz T,Lees A,de Silva R,Tsujihata M,Takahashi H

更新日期：2005-12-01 00:00:00

abstract：:Histomorphological and histochemical variability was studied in muscle specimens from 30 patients with congenital muscular dystrophy (CMD). We found involvement of the central nervous system in 8 patients (Fukuyama CMD, F-CMD), involvement of the brain and the eyes in 5 patients (muscle, eye and brain disease, MEB-D) ...

journal_title：Acta neuropathologica

authors： Leyten QH,ter Laak HJ,Gabreëls FJ,Renier WO,Renkawek K,Sengers RC

更新日期：1993-01-01 00:00:00

abstract：:Astrocytic alpha-synuclein-immunoreactive inclusions have recently been noted to develop in sporadic Parkinson's disease (PD). Here, the presence of immunoreactive astrocytes is reported in 14 autopsy cases with clinically diagnosed PD and a neuropathological stage of 4 or higher. The labeled astrocytes occur preferen...

journal_title：Acta neuropathologica

authors： Braak H,Sastre M,Del Tredici K

更新日期：2007-09-01 00:00:00

abstract：:Knockout of genes encoding metabotropic glutamate receptor 5 (mGluR5) or its endogenous regulators, such as Norbin, induce a schizophrenia-like phenotype in rodents, suggesting dysregulation of mGluR5 in schizophrenia. Human genetic and pharmacological animal studies support this hypothesis, but no studies have explor...

journal_title：Acta neuropathologica

authors： Matosin N,Fernandez-Enright F,Fung SJ,Lum JS,Engel M,Andrews JL,Huang XF,Weickert CS,Newell KA

更新日期：2015-07-01 00:00:00

abstract：:An unresolved issue in the study of demyelinating disease is whether blood-brain barrier damage is dependent upon the migration of inflammatory cells into the central nervous system (CNS). In a study of experimental autoimmune encephalomyelitis (EAE) in rabbits, a freeze-dried, paraffin-embedded tissue technique was e...

journal_title：Acta neuropathologica

authors： Simmons RD,Buzbee TM,Linthicum DS,Mandy WJ,Chen G,Wang C

更新日期：1987-01-01 00:00:00

abstract：:In the present study we assessed the neuroprotective effects of the pan-caspase inhibitor z-VAD.fmk [N-benzyloxycarbony-valine-alanine-aspartate-(OMe)-fluoromethylketone], and the caspase-3 inhibitor Ac-DEVD.CHO (acetyl-aspartate-chloromethylketone) in the double-lesion rat model of striatonigral degeneration (SND), t...

journal_title：Acta neuropathologica

authors： Mantoan L,Stefanova N,Egger KE,Jellinger KA,Poewe W,Wenning GK

更新日期：2005-02-01 00:00:00

abstract：:We have recently shown that glioma cell lines, as well as cells of human malignant gliomas in situ, synthesize tropoelastin. In addition, glioma cells degrade tropoelastin using metalloproteinase(s), and the resulting peptides, incapable of assembling in the extracellular fibers, interact with the 67-kDa cell surface ...

journal_title：Acta neuropathologica

authors： Hinek A,Jung S,Rutka JT

更新日期：1999-04-01 00:00:00

abstract：:Canine distemper virus (CDV) causes severe immunosuppression and neurological disease in dogs, associated with demyelination, and is a model for multiple sclerosis in man. In the early stage of the infection, demyelination is associated with viral replication in the white matter. In acute demyelinating lesions there i...

journal_title：Acta neuropathologica

authors： Vandevelde M,Zurbriggen A

更新日期：2005-01-01 00:00:00

abstract：:Peroxidase-labeled lectins were used for detection of specific monosaccharide residues in amyloid plaques in brains of scrapie-infected mice. The lectins tested recognize the following residues: beta-D-galactosyl (Ricinus communis agglutinin 120, RCA-1), alpha-D-galactosyl and alpha-D-galactopyranoside (Bandeirea simp...

journal_title：Acta neuropathologica

authors： Szumanska G,Vorbrodt AW,Wisniewski HM

更新日期：1986-01-01 00:00:00

abstract：:A histochemical study is reported of regional differences of the lipid storage in a case of Niemann-Pick disease (NPD) type C. Besides tissues known to be affected (reticulo-endothelium, hepatocytes, nervous system), storage was demonstrated in adrenal cortical spongiocytes, sweat glands, renal glomerular and tubular ...

journal_title：Acta neuropathologica

authors： Elleder M,Jirásek A,Smíd F

更新日期：1975-12-19 00:00:00

abstract：:Various parts of the central and peripheral nervous system of the mutant hamster with hind leg paralysis were studied in the electron microscope. A number of alterations involving the myelin sheath, the axon and certain neuronal perikarya were observed. Anterior horn cells were apparently well preserved. This conditio...

journal_title：Acta neuropathologica

authors： Hirano A

更新日期：1977-08-31 00:00:00

abstract：:Neuropathological studies have revealed that the brains of HIV-1-infected AIDS patients show the typical encephalitis and, in addition, neuronal loss. More recently, this neuronal cell loss has been thought to take place via programmed cell death (apoptosis) which has been demonstrated by an in situ end labelling (ISE...

journal_title：Acta neuropathologica

authors： An SF,Giometto B,Scaravilli T,Tavolato B,Gray F,Scaravilli F

更新日期：1996-01-01 00:00:00

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：:Uniform neuropathological changes are described in eight cases of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (PEHO syndrome). Two of the autopsied patients were sisters and two other cases were familial. Macroscopically, cerebral and pronounced cerebellar atrophy was seen, the...

journal_title：Acta neuropathologica

authors： Haltia M,Somer M

更新日期：1993-01-01 00:00:00