Progression in nemaline myopathy.

abstract：:Patients with von Hippel-Lindau disease carry a germline mutation of the Von Hippel-Lindau (VHL) tumor-suppressor gene. We discuss the molecular consequences of loss of VHL gene function and their impact on the nervous system. Dysfunction of the VHL protein causes accumulation and activation of hypoxia inducible facto...

journal_title：Acta neuropathologica

authors： Vortmeyer AO,Falke EA,Gläsker S,Li J,Oldfield EH

更新日期：2013-03-01 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：:Pelizaeus-Merzbacher disease (PMD) is an untreatable and fatal leukodystrophy. In a model of PMD with perturbed blood-brain barrier integrity, cholesterol supplementation promotes myelin membrane growth. Here, we show that in contrast to the mouse model, dietary cholesterol in two PMD patients did not lead to a major ...

journal_title：Acta neuropathologica

authors： Stumpf SK,Berghoff SA,Trevisiol A,Spieth L,Düking T,Schneider LV,Schlaphoff L,Dreha-Kulaczewski S,Bley A,Burfeind D,Kusch K,Mitkovski M,Ruhwedel T,Guder P,Röhse H,Denecke J,Gärtner J,Möbius W,Nave KA,Saher G

更新日期：2019-07-01 00:00:00

abstract：:We estimated the total neurone number, glial number, and glial index (ratio glial cells/neurone) in the thalamic mediodorsal nucleus (MD) in seven patients suffering from Huntington's disease (HD; four males, three females, mean age 52.4 +/- 13.6 years) and age- and sex-matched controls (four males, three females, mea...

journal_title：Acta neuropathologica

authors： Heinsen H,Rüb U,Bauer M,Ulmar G,Bethke B,Schüler M,Böcker F,Eisenmenger W,Götz M,Korr H,Schmitz C

更新日期：1999-06-01 00:00:00

abstract：:The occurrence of oligodendrocytes within astrocytes ("emperipolesis") has been described in demyelinating lesions in cases of multiple sclerosis and also in other non-demyelinating disorders. We found that this finding was common in the cerebral white matter of patients with Creutzfeldt-Jakob disease (CJD). Eight con...

journal_title：Acta neuropathologica

authors： Shintaku M,Yutani C

更新日期：2004-09-01 00:00:00

abstract：:In looking for a possible influence of nuclear inclusions (NIs) on neurodegeneration in human brains, we quantified morphological features of pontine neurons of three unrelated cases of neuronal intranuclear inclusion disease (NIID) and five control cases. Cross-sectional area of each neuronal nucleus and the indices ...

journal_title：Acta neuropathologica

authors： Uchihara T,Tanaka J,Funata N,Arai K,Hattori T

更新日期：2003-02-01 00:00:00

abstract：:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and a phenotypically similar recessive condition (CARASIL) have emerged as important genetic model diseases for studying the molecular pathomechanisms of cerebral small vessel disease (SVD). CADASIL, the most frequent ...

journal_title：Acta neuropathologica

authors： Zellner A,Scharrer E,Arzberger T,Oka C,Domenga-Denier V,Joutel A,Lichtenthaler SF,Müller SA,Dichgans M,Haffner C

更新日期：2018-07-01 00:00:00

abstract：:A 24-year-old woman was found comatose after 2 days of cephalalgia and vomiting. An immediate diagnosis of carbon monoxyde poisoning was disclaimed when blood carbon monoxyde was found to be 1.75 ml/100. A diagnosis of acute intracranial hypertension led to trephination with ventricular punction and brain biopsy on th...

journal_title：Acta neuropathologica

authors： Foncin JF,Le Beau J

更新日期：1978-08-07 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：: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

authors： Peltonen J,Näntö-Salonen K,Aho HJ,Kouri T,Virtanen I,Penttinen R

更新日期：1984-01-01 00:00:00

abstract：:Rats were given isoniazid either in a single large dose or continuously in drinking water and killed 5--105 days later. The distribution of degenerating fibres in various nerves (sensory and mixed) and in various sites along nerves and spinal roots was studied by light and electron microscopy. It was found that sensor...

journal_title：Acta neuropathologica

authors： Jacobs JM,Miller RH,Cavanagh JB

更新日期：1979-10-01 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

authors： Ozawa K,Saida T,Saida K,Nishitani H,Kameyama M

更新日期：1989-01-01 00:00:00

abstract：:Neuregulins belong to a family of multipotent growth-promoting proteins, and have been shown to have a crucial role in accumulating acetylcholine receptor at neuromuscular junctions. A functional role of neuregulins in muscle regeneration has not yet been identified. Using reverse transcription (RT)-PCR, Western blot ...

journal_title：Acta neuropathologica

authors： Hirata M,Sakuma K,Okajima S,Fujiwara H,Inashima S,Yasuhara M,Kubo T

更新日期：2007-04-01 00:00:00

abstract：:Mutations of isocitrate dehydrogenase 1 (IDH1) gene are most common in glioma, arguably preceding all known genetic alterations during tumor development. IDH1 mutations nearly invariably target the enzymatic active site Arg132, giving rise to the predominant IDH1R132H. Cells harboring IDH1 R132H -heterozygous mutation...

journal_title：Acta neuropathologica

authors： Tiburcio PDB,Xiao B,Berg S,Asper S,Lyne S,Zhang Y,Zhu X,Yan H,Huang LE

更新日期：2018-02-01 00:00:00

abstract：:The contribution of leukocyte apoptosis to the resolution of meningeal inflammation in bacterial meningitis was studied in the cerebrospinal fluid (CSF) and in meningeal infiltrates of humans and rabbits by in situ tailing, flow cytometry, agarose gel electrophoresis, and electron microscopy. In humans, the rate of ap...

journal_title：Acta neuropathologica

authors： Nau R,Zettl U,Gerber J,Trostdorf F,Michel U,Böttcher T,Schmidt H,Adler S,Brück W

更新日期：1998-11-01 00:00:00

abstract：:The expression of glial fibrillary acidic protein (GFAP) in continuous glioma and non-glioma cell lines was evaluated by molecular hybridization and immunocytochemistry. RNA transcripts from the GFAP gene were detected in two of six cell lines by Northern blot analysis of cellular RNAs using a cloned mouse GFAP cDNA p...

journal_title：Acta neuropathologica

authors： Dewhurst S,Stevenson M,McComb RD,Volsky DJ

更新日期：1987-01-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs), including FUS. How FUS aggregation and neurodegeneration are prevented in healthy motor neurons remain critically unanswered questions. Here, we use...

journal_title：Acta neuropathologica

authors： Marrone L,Drexler HCA,Wang J,Tripathi P,Distler T,Heisterkamp P,Anderson EN,Kour S,Moraiti A,Maharana S,Bhatnagar R,Belgard TG,Tripathy V,Kalmbach N,Hosseinzadeh Z,Crippa V,Abo-Rady M,Wegner F,Poletti A,Troost D,A

更新日期：2019-07-01 00:00:00

abstract：:Adult rabbits were subjected to 4.5 Gy of whole-blody or brain alone gamma-irradiation, and their hippocampus was examined with the light and electron microscope. Pycnotic cells were found at the base of the granular layer of the dentate gyrus in the so-called subgranular zone, as soon as 3 h after irradiation, and we...

journal_title：Acta neuropathologica

authors： Guéneau G,Drouet J,Privat A,Court L

更新日期：1979-12-01 00:00:00

abstract：:In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contai...

journal_title：Acta neuropathologica

authors： Oda M,Akagawa N,Tabuchi Y,Tanabe H

更新日期：1978-12-15 00:00:00

abstract：:The post-mortem diagnosis of lysosomal storage diseases can be confounded by the unavailability of suitable material. Here we report the diagnosis of GM1-gangliosidosis in a cross-bred dog, from which only formalin-fixed brain was available, by a combination of electron microscopy and the detection of elevated levels ...

journal_title：Acta neuropathologica

authors： Whitfield P,Johnson AW,Dunn KA,Delauche AJ,Winchester BG,Franklin RJ

更新日期：2000-10-01 00:00:00

abstract：:The immunohistochemical distribution of alpha and beta subunits of S-100 protein (S-100 alpha, S-100 beta, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100 alpha (+) and/or S-100 beta (+)]; astro...

journal_title：Acta neuropathologica

authors： Hayashi K,Hoshida Y,Horie Y,Takahashi K,Taguchi K,Sonobe H,Ohtsuki Y,Akagi T

更新日期：1991-01-01 00:00:00

abstract：:Cerebral β-amyloidosis can be exogenously induced by the intracerebral injection of brain extracts containing aggregated β-amyloid (Aβ) into young, pre-depositing Aβ precursor protein- (APP) transgenic mice. Previous work has shown that the induction involves a prion-like seeding mechanism in which the seeding agent i...

journal_title：Acta neuropathologica

authors： Fritschi SK,Cintron A,Ye L,Mahler J,Bühler A,Baumann F,Neumann M,Nilsson KP,Hammarström P,Walker LC,Jucker M

更新日期：2014-10-01 00:00:00

abstract：:Focal cerebral ischemia was induced by occlusion of the right middle cerebral artery in hypoglycemic, normoglycemic, as well as in acute and chronic diabetic rats. The brain damage was studied after 4 days. The volume of infarction was decreased in hypoglycemia (29 +/- 19 mm3 (mean +/- SD) versus 58 +/- 35 mm3, P less...

journal_title：Acta neuropathologica

authors： Nedergaard M,Diemer NH

更新日期：1987-01-01 00:00:00

abstract：:Missense mutations of the V600E type constitute the vast majority of tumor-associated somatic alterations in the v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) gene. Initially described in melanoma, colon and papillary thyroid carcinoma, these alterations have also been observed in primary nervous system tumors...

journal_title：Acta neuropathologica

authors： Schindler G,Capper D,Meyer J,Janzarik W,Omran H,Herold-Mende C,Schmieder K,Wesseling P,Mawrin C,Hasselblatt M,Louis DN,Korshunov A,Pfister S,Hartmann C,Paulus W,Reifenberger G,von Deimling A

更新日期：2011-03-01 00:00:00

abstract：:The present study used cocultures of rat dorsal root ganglia (DRG) and peritoneal macrophages to define the role of activated complement components during demyelination. The complement cascade was activated in vitro by treatment of the cultures with natural rat serum and lipopolysaccharides. Complement activation was ...

journal_title：Acta neuropathologica

authors： Brück W,Brück Y,Diederich U,Piddlesden SJ

更新日期：1995-01-01 00:00:00

abstract：:A complex neuropathological study of two cases of Niemann-Pick disease (NPD) type C (NPDC) revealed some novel features in the chemical pathology of the neuronal storage. Lipid histochemistry showed the presence of a lipid which met the criteria of a neuronal glycosphingolipid. Sphingomyelin (SM) was not detected in t...

journal_title：Acta neuropathologica

authors： Elleder M,Jirásek A,Smíd F,Ledvinová J,Besley GT

更新日期：1985-01-01 00:00:00

abstract：:In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30-600 microm in diameter. Some of these membrane-bound vesicular or tubular structure...

journal_title：Acta neuropathologica

authors： Schröder JM,Wang JF,Sindern E,Malin JP

更新日期：1999-10-01 00:00:00

abstract：:We report the case of an elderly woman with a history of headache, vomiting and dizziness while walking. On CT scans a mass was identified in the right cerebellar hemisphere exhibiting radiological characteristics of lipomatous tissue. Surgery revealed a compact lesion consisting of whitish-yellow tissue with a fatty ...

journal_title：Acta neuropathologica

authors： Walter A,Dingemans KP,Weinstein HC,Troost D

更新日期：1994-01-01 00:00:00

abstract：:The pattern of recovery of myelinated axons in the posterior tibial nerve after crushing was studied in rats chronically intoxicated with 2,5-hexanedione. It was given for 2 weeks before crushing (200 mg/kg i.p. 5 times a week) or additionally for two further weeks after the nerve crush. Two animals were examined from...

journal_title：Acta neuropathologica

authors： Simonati A,Rizzuto N,Cavanagh JB

更新日期：1983-01-01 00:00:00

abstract：:The quadriceps femoris muscles of experimental allergic myositis, in strain 13 guinea pigs immunised with rabbit myosin B fraction, were subjected to histochemical, immunohistochemical and electron microscopic studies. They demonstrated a variety of degenerative changes of muscle fibres, infiltration of lymphocytes an...

journal_title：Acta neuropathologica

authors： Matsubara S,Takamori M

更新日期：1987-01-01 00:00:00