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1
المؤلفون: Sarah K. Fritschi, Philipp J. Kahle, Manuel Schweighauser, Yvonne S. Eisele, Derya R. Shimshek, Mathias Jucker, Mehtap Bacioglu
المصدر: Acta neuropathologica 129(1), 157-159 (2014). doi:10.1007/s00401-014-1360-5
مصطلحات موضوعية: Genetically modified mouse, pathology [Dentate Gyrus], Tissue Fixation, Neurite, Transgene, physiopathology [Brain Stem], Immunoblotting, Peptide, Mice, Transgenic, Brain tissue, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, pathology [Brain Stem], pathology [Proteostasis Deficiencies], Formaldehyde, mental disorders, physiopathology [Proteostasis Deficiencies], Amyloid precursor protein, medicine, Animals, physiopathology [Dentate Gyrus], ddc:610, metabolism [alpha-Synuclein], SNCA protein, human, Proteostasis Deficiencies, chemistry.chemical_classification, biology, Chemistry, Dementia with Lewy bodies, Tissue Extracts, physiopathology [Neurodegenerative Diseases], pathology [Neurodegenerative Diseases], Neurodegenerative Diseases, Snca protein, mouse, medicine.disease, Molecular biology, Immunohistochemistry, Survival Analysis, nervous system diseases, Dentate Gyrus, biology.protein, genetics [alpha-Synuclein], alpha-Synuclein, α synuclein, Female, Neurology (clinical), Brain Stem
الوصف: attributes of prions, namely the resistance to inactivation by formaldehyde. The histopathological hallmarks of α-synucleinopathies such as Parkinson’s disease (PD) and dementia with Lewy bodies (DLB) are intracellular Lewy bodies and Lewy neurites, comprised primarily of hyperphosphorylated α-synuclein [3]. In PD brain, α-synuclein lesions progress in a stereotypic manner [1]. The underlying mechanism is hypothesized to be cell-to-cell transmission of aggregated α-synuclein that initiates a cascade of progressive α-synuclein misfolding and aggregation reminiscent of prion disorders [4, 5]. Experimentally similar to Aβ inoculations, α-synuclein lesions can be induced in susceptible hosts by intracerebral inoculation of extracts from human brains affected by α-synucleinopathies or brains from spontaneously ill α-synuclein tg mice containing aggregated α-synuclein [6, 7]. Given the astonishing findings of formaldehyde-resistant Aβ seeds [2], we asked whether this is also true for α-synuclein seeds. Compelling evidence suggests that in a variety of neurodegenerative diseases the induction and spreading of proteinaceous lesions involve a prion-like seeding mechanism [5]. Experimentally and for Alzheimer’s disease (AD), it has been shown that cerebral β-amyloidosis can be instigated in susceptible hosts [i.e., young amyloid precursor protein (APP) transgenic (tg) mice] by the intracerebral injections of diluted extracts from β-amyloid-laden brains of aged APP tg mice or AD patients. The β-amyloid-inducing agent in the inoculate is an aggregated form of the amyloid-β peptide (Aβ) [8]. Remarkably, we recently reported that extracts of formaldehyde-fixed brains of aged APP tg mice or AD patients also induces cerebral β-amyloidosis [2]. Thus, Aβ seeds share one of the most remarkable
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2
المؤلفون: Johannes Attems, Kurt A. Jellinger, Magdalena Quass
المصدر: Acta Neuropathologica. 113:53-62
مصطلحات موضوعية: Male, Pathology, medicine.medical_specialty, tau Proteins, Substantia nigra, Statistics, Nonparametric, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, chemistry.chemical_compound, Basal Ganglia Diseases, Extrapyramidal symptoms, Alzheimer Disease, Humans, Medicine, Pathological, Aged, Retrospective Studies, Aged, 80 and over, Psychiatric Status Rating Scales, Alpha-synuclein, business.industry, Middle Aged, medicine.disease, nervous system, chemistry, Nerve Degeneration, alpha-Synuclein, Medulla oblongata, Locus coeruleus, Female, Neurology (clinical), Brainstem, medicine.symptom, Alzheimer's disease, business, Brain Stem
الوصف: Extrapyramidal symptoms (EPS) in Alzheimer disease (AD) often increase with disease severity. Their neuropathological substrate is a matter of discussion. We investigated tau and α-synuclein (AS) pathologies in brainstem in AD patients with and without EPS. Among 160 elderly subjects with autopsy-proven AD (110 female, 50 male, aged 61–102, mean 84.1 ± 8.3 SD years), 151 (94.4%) being demented, 35 (21.9%) had clinically reported EPS (rigidity, bradykinesia, gait impairment). Neuropathological examination included standardized classification of AD according to current criteria, and semiquantitative assessment of neuronal loss in substantia nigra (SN), locus coeruleus (LC), and of tau and AS lesions in brainstem, and, in addition, of cerebrovascular lesions. The prevalence of EPS was only slightly more frequent in higher Braak stages. Tau pathology in brainstem significantly increased with increasing Braak stages, while AS lesions did not. EPS correlated best with SN cell loss (P
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3
المؤلفون: Hans A. Kretzschmar, Silke Adler, Reiner Benecke, Oliver M. Schlüter, Manuela Neumann, Elisabeth Kremmer
المصدر: Acta neuropathologica. 100(5)
مصطلحات موضوعية: Adult, Pathology, medicine.medical_specialty, Neurodegeneration with brain iron accumulation, Iron, Synucleins, Substantia nigra, Nerve Tissue Proteins, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, chemistry.chemical_compound, Atrophy, medicine, Humans, Pantothenate Kinase-Associated Neurodegeneration, Alpha-synuclein, Cerebral Cortex, Lewy body, Dementia with Lewy bodies, business.industry, Brain, medicine.disease, Immunohistochemistry, Magnetic Resonance Imaging, nervous system diseases, Microscopy, Electron, medicine.anatomical_structure, Globus pallidus, nervous system, chemistry, Cerebral cortex, Nerve Degeneration, alpha-Synuclein, Female, Lewy Bodies, Neurology (clinical), business, Brain Stem
الوصف: 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-weighted images, supporting the clinical diagnosis of neurodegeneration with brain iron accumulation type 1 (NBIA-1; formerly known as Hallervorden-Spatz syndrome). At autopsy the brain showed mild frontotemporal atrophy and discoloration of the globus pallidus and the substantia nigra pars reticularis. Histologically, features typical of NBIA-1 were found including widespread axonal spheroids and large deposits of iron pigment in the discolored regions. Additionally, excessive numbers of Lewy bodies (LBs) were found throughout all examined brain stem and cortical regions. LBs of both types, as well as Lewy neurites in this case of NBIA-1, were strongly labeled by antibodies against alpha-synuclein. These findings give further evidence that accumulation of alpha-synuclein is generally associated with LB formation, i.e., in Parkinson's disease, dementia with Lewy bodies and NBIA-1. The case presented here is particularly notable for its high number of LBs in all areas of the cerebral cortex.
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4
المؤلفون: Y. Izumiyama, N. Horita, T. Ishii
المصدر: Acta Neuropathologica. 49:19-27
مصطلحات موضوعية: Central Nervous System, Male, Niacinamide, Aging, Pathology, medicine.medical_specialty, Central nervous system, Encephalopathy, Striatum, Biology, Creutzfeldt-Jakob Syndrome, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, chemistry.chemical_compound, Central Nervous System Diseases, Pellagra, Limbic System, medicine, Animals, Humans, Cerebral Cortex, Neurons, Nicotinamide, medicine.disease, Corpus Striatum, Rats, 6-Aminonicotinamide, medicine.anatomical_structure, Spinal Cord, chemistry, Cerebral cortex, Chromatolysis, Neurology (clinical), NAD+ kinase, Brain Stem
الوصف: Lesions in the CNS induced by 6-aminonicotinamide (6-AN) presented a spongy state of the gray matter and neuronal chromatolysis. With aging of the experimental animals the lesions extended from the phylogenetically early developed structures to those developed later, i.e., from spinal gray matter, dentate nuclei, and brain stem nuclei through limbic structures and striatum to the cerebral cortex. Changes of the neurons were more prominent with aging. Lesions in the CNS of rats at the age, corresponding to the involutional period in the human, were similar to those of Creutzfeldt-Jakob disease (C-J) disease) in the presenile age. In recent years, the resemblance between C-J disease and pellagra encephalopathy had been noted by several authors, and they resemble the lesions caused by 6-AN, an antimetabolite of nicotinamide used in our experiment. This evidence, therefore, has led to the hypothesis that dysfunction of NAD(H)- or NADP(H)-dependent enzymes in the CNS of the aged, even if not the primary cause, may be one possible pathogenetic factor of C-J disease.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::86ba1e864fc22acf3b7506afa074e2d8
https://doi.org/10.1007/bf00692215 -
5
المؤلفون: P. Gambetti, Alberto Bizzi
المصدر: Acta Neuropathologica. 71:154-158
مصطلحات موضوعية: Hypoglossal Nerve, Neurofilament, medicine.drug_class, Phosphatase, Monoclonal antibody, Axonal Transport, Epitope, Pathology and Forensic Medicine, Immunoenzyme Techniques, Cellular and Molecular Neuroscience, Intermediate Filament Proteins, medicine, Animals, Phosphorylation, biology, Chemistry, Molecular biology, nervous system, Biochemistry, Axoplasmic transport, biology.protein, Rabbits, Neurology (clinical), Antibody, Immunostaining, Aluminum, Brain Stem
الوصف: A series of monoclonal antibodies that distinguish phosphorylated and nonphosphorylated neurofilament (NF) epitopes was used to immunostain brain stem neurons from control rabbits and from rabbits chronically intoxicated with Aluminium (Al). In controls, none of the monoclonal antibodies to phosphorylated NF stained the perikaryon of neurons. In contrast, in animals treated with Al, all neuronal perikarya containing Al-induced neurofilament bundles (NB) and some lacking well-formed NB immunoreacted with two of the five antibodies to phosphorylated NF. Axons were stained by all five antibodies to phosphorylated NF in both control and Al-treated animals. A broadly reacting monoclonal antibody to a nonphosphorylated NF epitopes immunoreacted with neuronal cell bodies, dendrites and axons in control and Al-intoxicated animals regardless of the presence of Al-induced NB. Staining of Al-induced NB with one of the antibodies to phosphorylated NF was greatly diminished after treatment of sections with trypsin and phosphatase. It is concluded that NF which compose the Al-induced NB have different immunocytochemical characteristics from those of the NF present in the perikaryon of normal neurons. It is likely that, contrary to normal perikaryal NF, NF of Al-induced NB are phosphorylated. Moreover, phosphorylation of NF of Al-induced NB is probably abnormal, since NF of Al-induced NB have immunostaining characteristics different from NF of normal axons. Al-induced NB may result from abnormal phosphorylation of NF in the perikaryon, preventing normal axonal transport of these structures.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f80996d9b7b2f480d1242a7c600981ec
https://doi.org/10.1007/bf00687978 -
6
المؤلفون: H. Schneider, J. Cervos-Navarro
المصدر: Acta Neuropathologica. 27:11-23
مصطلحات موضوعية: Male, Niacinamide, Cytoplasm, Pathology, medicine.medical_specialty, Necrosis, medicine.drug_class, Hemorrhage, Biology, Endoplasmic Reticulum, Antimetabolite, Spinal Cord Diseases, Pathology and Forensic Medicine, Pathogenesis, Cellular and Molecular Neuroscience, chemistry.chemical_compound, Myelopathy, Cerebellum, medicine, Animals, Cell Nucleus, Medulla Oblongata, Nicotinamide, Microcirculation, Reticular Formation, Endoplasmic reticulum, Cranial Nerves, Anatomy, Spinal cord, medicine.disease, Rats, Microscopy, Electron, medicine.anatomical_structure, Spinal Cord, chemistry, Cats, Focal necrosis, Female, Neurology (clinical), medicine.symptom, Neuroglia, Injections, Intraperitoneal, Brain Stem
الوصف: 6-aminonicotinamide, a potent antimetabolite of nicotinamide, produces, in lower doses of 5–10 mg/kg, selective damage of neuroglial cells in the gray matter of spinal cord and lower brain stem of the rat. Oligodendrocytes and astrocytes exhibit progredient vacuolisation and destruction of the rough endoplasmic reticulum, dilatation of the perinuclear cistern, and necrosis within 36 h after administration of the drug. The disintegration of the astroglial processes initiates the incomplete necrosis of the central anterior horn of the spinal cord. Since the neurons are less involved in the affected areas (“pseudomalacia”), it can be concluded that the biosynthesis of abnormal 6-ANAD(P)-analogues takes place mainly in neuroglial cells. The relationship between structural lesions and metabolic disturbances, and the pathogenesis of status spongiosus and focal necrosis are discussed in this model of experimental myelopathy.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9ac367927ce150f83e70770e827f4d09
https://doi.org/10.1007/bf00687236 -
7
المؤلفون: Nicholas A. Vick, Nathan K. Blank, Sidney Schulman
المصدر: Acta Neuropathologica. 31:137-150
مصطلحات موضوعية: Pathology, medicine.medical_specialty, Encephalopathy, Striatum, Pathology and Forensic Medicine, Wernicke's encephalopathy, Lesion, Necrosis, Cellular and Molecular Neuroscience, Myelin, medicine, Neuropil, Animals, Wernicke Encephalopathy, Myelin Sheath, Chemistry, Thiamine Deficiency, medicine.disease, Macaca mulatta, Axons, Corpus Striatum, Capillaries, Mitochondria, Disease Models, Animal, medicine.anatomical_structure, nervous system, Astrocytes, Vacuoles, Ultrastructure, Thiamine, Neurology (clinical), medicine.symptom, Brain Stem, Demyelinating Diseases
الوصف: Experimental Wernicke's encephalopathy, induced in rhesus monkeys with a diet lacking thiamine (vitamin B1), is characterized by cavitary necrosis of the striatum as well as a microvacuolar periventricular lesion of the brain stem such as occurs in man. With high resolution light microscopy and electron microscopy, the primary structural alteration in the brain stem lesion, and probably also in the striatum, appears to be that of widespread "blister" formation due to splitting of myelin at the intraperiod line. Microvascular alterations were minimal, even in the most severely affected regions. It is the myelin blisters which give rise to the spongy texture of the neuropil. A similar splitting of myelin has been described in several other experimental encephalopathies, and it is probable that it also occurs in Wernicke's encephalopathy in man.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a9ca8051cf45e5d6a3955d116334eaa2
https://doi.org/10.1007/bf00688148 -
8
المؤلفون: N. Kopp, L. Denoroy, M. Tommasi, N. Gay, G. Chazot, B. Renaud
المصدر: Acta Neuropathologica. 56:17-21
مصطلحات موضوعية: Male, endocrine system, medicine.medical_specialty, Nucleus tractus solitarius, Parkinson's disease, Dopamine beta-Hydroxylase, Pathology and Forensic Medicine, Parkinsonian syndromes, Hypotension, Orthostatic, Cellular and Molecular Neuroscience, Internal medicine, medicine, Humans, Medulla, Aged, chemistry.chemical_classification, Medulla Oblongata, biology, business.industry, Phenylethanolamine N-Methyltransferase, Parkinson Disease, Middle Aged, medicine.disease, Enzyme assay, Dihydroxyphenylalanine, Endocrinology, Enzyme, nervous system, chemistry, Catecholamine, Medulla oblongata, biology.protein, Female, Neurology (clinical), business, Brain Stem, medicine.drug
الوصف: Dopamine beta hydroxylase (DBH), The noradrenaline-synthesizing enzyme, and phenyl-ethanolamine-N-methyltransferase (PNMT), the adrenaline-synthesizing enzyme, were assayed in 18 areas of brain stem in eight cases of parkinsonian syndromes and of four age- and postmortem delay-matched controls. Dissection was performed by the "punch" technique and enzyme activities assayed by radiometric methods. No significant change was found for PNMT activity. DBH activity was significantly increased in the A2-C2 area of the medulla oblongata (including the nucleus tractus solitarius) in the cases of Parkinson's disease. The A2-C2 area is known to be implicated in the control of blood pressure in rats. These findings are discussed in relation to orthostatic hypotension and the influence of L-dopa therapy.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cc234c080bc70b29b215fa618a70b53b
https://doi.org/10.1007/bf00691177 -
9
المؤلفون: Ralph M. Garruto, Richard Yanagihara, Salazar Am, S. K. Shankar, H L Amyx, D. C. Gajdusek
المصدر: Acta Neuropathologica. 78:210-219
مصطلحات موضوعية: Pathology, medicine.medical_specialty, Neurofilament, Intermediate Filaments, chemistry.chemical_element, Substantia nigra, Biology, Calcium, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, medicine, Animals, Amyotrophic lateral sclerosis, Motor Neurons, Cerebrum, Manganese Poisoning, Amyotrophic Lateral Sclerosis, Motor neuron, Spinal cord, medicine.disease, Diet, Calcium, Dietary, Macaca fascicularis, medicine.anatomical_structure, Spinal Cord, chemistry, Chromatolysis, Neurology (clinical), Aluminum, Brain Stem
الوصف: 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 magnesium and high levels of aluminum in the soil and drinking water, along with the relative isolation of these populations, constitute an unusual environmental feature common to all three high-incidence foci. Studies of mineral deposition in brain tissue of Guamanian ALS and PD patients, as well as of neurologically normal Guamanians with neurofibrillary degeneration, demonstrate accumulations of calcium, aluminum and silicon in neurofibrillary tangle-bearing neurons. In an attempt to duplicate the low calcium and high aluminum and manganese in soil and drinking water in these foci, we maintained juvenile cynomolgus monkeys for 41 to 46 months on a low-calcium diet with or without supplemental aluminum and manganese. Experimental animals exhibited mild calcium and aluminum deposition and degenerative changes, compatible with those of early ALS and PD, in motor neurons of the spinal cord, brain stem, substantia nigra and cerebrum. Neuropathological findings included chromatolysis, aberrant perikaryal accumulation of phosphorylated neurofilament, neurofibrillary tangles, axonal spheroids, and basophilic and hyaline-like inclusions consisting of abnormal cytoskeletal elements by electron microscopy. The magnitude and extent of these lesions far exceeded those found in normal aged monkeys.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9bcdff95159b2f1bc311052ae7130665
https://doi.org/10.1007/bf00688211 -
10
المؤلفون: Samuel K. Ludwin, N. H. Sternberger
المصدر: Acta Neuropathologica. 63:240-248
مصطلحات موضوعية: Male, Pathology, medicine.medical_specialty, Peduncle (anatomy), Central nervous system, Pathology and Forensic Medicine, Immunoenzyme Techniques, Mice, Cellular and Molecular Neuroscience, Myelin, Cerebellum, medicine, Animals, Axon, Remyelination, Myelin Sheath, chemistry.chemical_classification, Myelin-associated glycoprotein, biology, Myelin Basic Protein, Axons, Nerve Regeneration, Myelin basic protein, Myelin-Associated Glycoprotein, Oligodendroglia, medicine.anatomical_structure, nervous system, chemistry, Immunology, biology.protein, Neurology (clinical), Glycoprotein, Myelin Proteins, Brain Stem
الوصف: The process of remyelination in the superior cerebellar peduncles of mice following demyelination with Cuprizone was studied immunohistochemically using antisera to myelin basic protein (MBP) and myelin-associated glycoprotein (MAG). Demyelination occurred after formation of myelinic vacuoles and resulted in almost complete loss of demonstrable MBP and MAG from the peduncle. Prior to the onset of remyelination, oligodendrocytes with cytoplasmic staining for both proteins appeared in the peduncle. These cells were then associated with remyelinating axons. The axons were remyelinated in clusters until the MBP and the MAG in the whole peduncle were reconstituted, although the axon sheaths were thinner than those in normal animals. The results show that the immunohistochemical distribution of MBP and MAG in remyelinating axons resembles that in normal axons, and that the expression of myelin proteins in oligodendrocytes during remyelination reverts to that seen during normal development.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::485cc6a1bc2b9bdaa9806ec18ee0ba64
https://doi.org/10.1007/bf00685250
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