The function of the cellular prion protein in health and disease.

abstract：:A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth c...

journal_title：Acta neuropathologica

authors： Barth PG,Stam FC,von der Harten JJ

更新日期：1978-04-26 00:00:00

abstract：:Zitter mutant rats exhibit abnormal metabolism of superoxide species and demonstrate progressive degeneration of dopamine (DA) neurons in the substantia nigra (SN). Furthermore, long-term intake of vitamin E, an effective free radical scavenger, prevents the loss of DA neurons caused by free radicals. However, it is u...

journal_title：Acta neuropathologica

authors： Nakadate K,Noda T,Sakakibara S,Kumamoto K,Matsuura T,Joyce JN,Ueda S

更新日期：2006-07-01 00:00:00

abstract：:Mineralization in the wall of central nervous system blood vessels is sporadically encountered in aged horses and cattle as in man, generally as an age-related change. This phenomenon has not to date been located in the meninges in dogs or cats. The present study reports a retrospective histological examination of 50 ...

journal_title：Acta neuropathologica

authors： Mandara MT

更新日期：2003-03-01 00:00:00

abstract：:Progressive myopathy developed in two women who consumed ipecac syrup containing emetine hydrochloride to induce vomiting as part of their anorexia nervosa. Muscle biopsy specimens were characterized by severe disruption of the sarcomeres. The ultrastructural spectrum extended from "Z-band streaming" to the formation ...

journal_title：Acta neuropathologica

authors： Halbig L,Gutmann L,Goebel HH,Brick JF,Schochet S

更新日期：1988-01-01 00:00:00

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 ...

journal_title：Acta neuropathologica

authors： Jeffrey M,McGovern G,Sisó S,González L

更新日期：2011-01-01 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：:To identify antigenic differences between gliomas and normal brain, we have immunohistochemically studied the expression of lymphocyte adhesion molecules (ICAM-1, ICAM-2, ICAM-3, VCAM-1, E-selectin and CD58), epidermal growth factor receptor (EGFR) and extracellular matrix proteins (collagen IV, fibronectin, laminin, ...

journal_title：Acta neuropathologica

authors： Mäenpää A,Kovanen PE,Paetau A,Jäáskeläinen J,Timonen T

更新日期：1997-09-01 00:00:00

abstract：:A 56-day-old infant with alpha-neuraminidase deficiency, whose clinical features included severe edema of extremities and ascites which resembled those in severe infantile sialidosis, was autopsied. Perforation, whose pathogenesis was unclear, was found on the descending portion of the duodenum. Light and electron mic...

journal_title：Acta neuropathologica

authors： Yamano T,Shimada M,Matsuzaki K,Matsumoto Y,Yoshihara W,Okada S,Inui K,Yutaka T,Yabuuchi H

更新日期：1986-01-01 00:00:00

abstract：:Pilocytic astrocytomas (PAs) are the most common brain tumors in pediatric patients and can cause significant morbidity, including chronic neurological deficiencies. They are characterized by activating alterations in the mitogen-activated protein kinase pathway, but little else is known about their development. To ma...

journal_title：Acta neuropathologica

authors： Lambert SR,Witt H,Hovestadt V,Zucknick M,Kool M,Pearson DM,Korshunov A,Ryzhova M,Ichimura K,Jabado N,Fontebasso AM,Lichter P,Pfister SM,Collins VP,Jones DT

更新日期：2013-08-01 00:00:00

abstract：:Neuropeptides have turned out to be promising new parameters, in addition to the routinely performed histochemical diagnosis, of Hirschsprung's disease (HD). Studies of the peptidergic innervation of the affected intestinal segment of patients with HD have demonstrated a marked reduction in the density of several neur...

journal_title：Acta neuropathologica

authors： Berger A,Kofler B,Santic R,Zipperer E,Sperl W,Hauser-Kronberger C

更新日期：2003-01-01 00:00:00

abstract：:Histamine dihydrochloride (10 micrograms of 500 micrograms/ml) was infused during 1 min into the lateral cerebral ventricle of rats, which resulted in a significant stimulation of pinocytosis in the endothelial cells. Systemic injections of mepyramine or metiamide could not prevent this activation. In contrast, raniti...

journal_title：Acta neuropathologica

authors： Dux E,Dóczi T,Joó F,Szerdahelyi P,Siklós L

更新日期：1988-01-01 00:00:00

abstract：:Spinal and bulbar muscular atrophy (SBMA) is an inherited neuromuscular disease caused by expansion of a polyglutamine (polyQ) tract in the androgen receptor (AR). SBMA is triggered by the interaction between polyQ-AR and its natural ligands, testosterone and dihydrotestosterone (DHT). SBMA is characterized by the los...

journal_title：Acta neuropathologica

authors： Malena A,Pennuto M,Tezze C,Querin G,D'Ascenzo C,Silani V,Cenacchi G,Scaramozza A,Romito S,Morandi L,Pegoraro E,Russell AP,Sorarù G,Vergani L

更新日期：2013-07-01 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：:Olfactory dysfunction is a frequent and early feature of patients with neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD) and is very uncommon in patients with frontotemporal dementia (FTD). Mechanisms underlying this clinical manifestation are poorly understood but the premature...

journal_title：Acta neuropathologica

authors： Mundiñano IC,Caballero MC,Ordóñez C,Hernandez M,DiCaudo C,Marcilla I,Erro ME,Tuñon MT,Luquin MR

更新日期：2011-07-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have identified several new molecular constituents of ALS-linked cellular aggregates, including FUS, TDP-43, OPTN, UBQLN2 and the translational product...

journal_title：Acta neuropathologica

authors： Blokhuis AM,Groen EJ,Koppers M,van den Berg LH,Pasterkamp RJ

更新日期：2013-06-01 00:00:00

abstract：:We studied three siblings and one unrelated patient with cerebrotendinous xanthomatosis (CTX). Of two unrelated patients, we examined biopsies of sural nerve, soleus muscle, and achilles tendon. We also performed neurophysiologic investigations. Another patient died, and a postmortem examination of both brain and spin...

journal_title：Acta neuropathologica

authors： Pop PH,Joosten E,van Spreeken A,Gabreëls-Festen A,Jaspar H,ter Laak H,Vos A

更新日期：1984-01-01 00:00:00

abstract：:Neurons of Area 11 in the fronto-orbital cortex of 18 unselected AIDS brains are analyzed by means of stereology. Neurological abnormalities including dementing symptoms were described in eight patients. Neuropathology diagnosed human immunodeficiency virus (HIV)-specific changes in four, and diffuse poliodystrophy in...

journal_title：Acta neuropathologica

authors： Ketzler S,Weis S,Haug H,Budka H

更新日期：1990-01-01 00:00:00

abstract：:An experiment was designed to examine the course of degeneration, phagocytosis, and regeneration in the central nervous system following surgical deafferentation. The anterior cerebellar vermis was ablated in young male rats. The animals were sacrificed by perfusion at postoperative times ranging from 24 hrs to 6 mont...

journal_title：Acta neuropathologica

authors： Johnson JE Jr

更新日期：1975-12-19 00:00:00

abstract：:In this study we evaluated the relationship between polyglucosan bodies and peripheral nerve lesions. The biopsied sural nerve from a patient with late-onset chronic sensori-motor neuropathy showed many intra-axonal polyglucosan bodies and segmental demyelination/remyelination. The formation of Schwann cell hyperplasi...

journal_title：Acta neuropathologica

authors： Matsumuro K,Izumo S,Minauchi Y,Inose M,Higuchi I,Osame M

更新日期：1993-01-01 00:00:00

abstract：:It is conventionally accepted that during the early stages of Wallerian degeneration of myelinated peripheral nerve fibres Schmidt-Lanterman incisures represent the sites at which the myelin sheath, together with enclosed axoplasm, is segmented into myelin ovoids. This mechanism is considered by some authors to be fac...

journal_title：Acta neuropathologica

authors： Ghabriel MN,Allt G

更新日期：1979-11-01 00:00:00

abstract：:A case of 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cereb...

journal_title：Acta neuropathologica

authors： Kleinert R,Beham A,Rosanelli G

更新日期：1985-01-01 00:00:00

abstract：:We describe three cases of early- (cases 1-3, 28-39 years) and one of late-onset (case 4, 76 years) Alzheimer's disease (AD) with 'cotton wool' plaques (CWPs) but without a family history indicating autosomal dominant inheritance. The early-onset cases, but not the late-onset case, showed remarkable aggression, disinh...

journal_title：Acta neuropathologica

authors： Yokota O,Terada S,Ishizu H,Ujike H,Ishihara T,Namba M,Hayashi Y,Nishinaka T,Namba R,Nakashima H,Uéda K,Checler F,Kuroda S

更新日期：2003-10-01 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

authors： Kawano N,Ohba Y,Nagashima K

更新日期：2000-02-01 00:00:00

abstract：:We report a patient who showed pathological features of both multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) at autopsy. The clinical features included severe cerebellar ataxia, autonomic failure, and rigid-akinetic parkinsonism. The clinical diagnosis was MSA. Pathological examination showed se...

journal_title：Acta neuropathologica

authors： Takanashi M,Ohta S,Matsuoka S,Mori H,Mizuno Y

更新日期：2002-01-01 00:00:00

abstract：:Abnormal TDP-43 aggregation is a prominent feature in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in TARDBP, the gene encoding TDP-43, cause some cases of ALS. The normal function of TDP-43 remains incompletely understood. To better understand TDP-43 biolo...

journal_title：Acta neuropathologica

authors： Kraemer BC,Schuck T,Wheeler JM,Robinson LC,Trojanowski JQ,Lee VM,Schellenberg GD

更新日期：2010-04-01 00:00:00

abstract：:Collections of human postmortem brains gathered in brain banks have underpinned many significant developments in the understanding of central nervous system (CNS) disorders and continue to support current research. Unfortunately, the worldwide decline in postmortem examinations has had an adverse effect on research ti...

journal_title：Acta neuropathologica

authors： Bell JE,Alafuzoff I,Al-Sarraj S,Arzberger T,Bogdanovic N,Budka H,Dexter DT,Falkai P,Ferrer I,Gelpi E,Gentleman SM,Giaccone G,Huitinga I,Ironside JW,Klioueva N,Kovacs GG,Meyronet D,Palkovits M,Parchi P,Patsouris E,

更新日期：2008-05-01 00:00:00

abstract：:The isolated paired helical filaments (PHF) that occur in the neurofibrillary tangles of Alzheimer's disease were assayed to determine if they contained N-acetyl-glucosamine and N-acetyl-galactosamine residues. The enzyme-linked lectin assay was used to detect their total content in the PHF preparation. The assay empl...

journal_title：Acta neuropathologica

authors： Sparkman DR,Hill SJ,White CL 3rd

更新日期：1990-01-01 00:00:00

abstract：:The amyloid cascade hypothesis of Alzheimer's disease (AD) is testable: it implies that interference with Abeta aggregation and plaque formation may be therapeutically useful. Abeta42 immunisation of amyloid precursor protein (APP) transgenic mice prevented plaque formation and caused removal of existing plaques. The ...

journal_title：Acta neuropathologica

authors： Boche D,Denham N,Holmes C,Nicoll JA

更新日期：2010-09-01 00:00:00

abstract：:Two cases of Central neurocytoma arising in the lateral ventricles are presented. Both patients had well-circumscribed masses in the right lateral ventricle causing hydrocephalus. The tumors were composed of small round cells forming Homer Wright rosettes against a fine fibrillary background. In one patient surgical r...

journal_title：Acta neuropathologica

authors： Townsend JJ,Seaman JP

更新日期：1986-01-01 00:00:00

abstract：:An in vivo method for positively staining dead neurons was developed and compared with an in vitro staining method using acid fuchsin. Neurons previously killed by intracerebral injections of kainic acid were selectively stained by trypan blue within 15 min of its injection in vivo into the central nervous system of r...

journal_title：Acta neuropathologica

authors： Lees GJ

更新日期：1989-01-01 00:00:00