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 for its deformity (long axis/short axis and circularity index defined as deviation from the perfect circle) were measured on pontine sections and their relation to NIs was statistically analyzed. Cross-sectional area of neuronal nuclei harboring ubiquitin-immunopositive NIs was significantly larger (110.6+/-1.6 micro m(2), mean +/- SE), while that of nuclei not harboring NIs was smaller (77.8+/-1.5 micro m(2)) than that in controls (90.5+/-0.7 micro m(2)). This difference remained significant even when the cross-sectional area occupied by NIs was subtracted from that of the nucleus harboring the NI (97.4+/-1.5 micro m(2)). This could hardly be explained if nuclear shrinkage is accelerated in the presence of NI. On the contrary, NI formation in pontine neurons of NIID might be linked, either directly or indirectly, to a mechanism, which counteracts rather than accelerates nuclear shrinkage. Because nuclear deformity was apparent even in neurons with NIs, whose nuclei were significantly larger than controls, the nuclear deformity is not secondary to its shrinkage and represents another aspect of neurodegeneration independent of nuclear shrinkage. Association of NIs to neurons of larger nuclear size in NIID brain indicates that NIs are not necessarily toxic to neurons.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Uchihara T,Tanaka J,Funata N,Arai K,Hattori Tdoi
10.1007/s00401-002-0614-9subject
Has Abstractpub_date
2003-02-01 00:00:00pages
103-8issue
2eissn
0001-6322issn
1432-0533journal_volume
105pub_type
杂志文章abstract::We described the first two unrelated Polish families with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). In the morphological examination with light microscopy, two kinds of changes were observed: (1). panarteritis nodosa-like changes with eosinophilic fibrinoid n...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0764-4
更新日期:2003-12-01 00:00:00
abstract::A monoclonal antibody, raised against extracts from Alzheimer brain, that recognizes a phosphorylated epitope in high molecular weight neurofilament proteins and tau proteins also immunostains Alzheimer neurofibrillary tangles, neurites in senile plaques and granulovacuolar degeneration. This result suggest that granu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686619
更新日期:1987-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00687981
更新日期:1986-01-01 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
pub_type: 杂志文章
doi:10.1007/s00401-002-0613-x
更新日期:2003-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00703206
更新日期:1983-01-01 00:00:00
abstract::Several cases of progressive multifocal leukoencephalopathy (PML) have been associated with simian virus 40 (SV40), rather than with JC virus (JCV), the polyomavirus originally isolated from PML tissue. PML has, therefore, been defined as a demyelinating syndrome with possible multiple viral etiologies. Tissues from t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050894
更新日期:1998-09-01 00:00:00
abstract::Foetal rat brain reaggregate cultures have been employed to investigate the morphological changes associated with the neurotoxic action of ethylcholine mustard aziridinium (ECMA). In a companion study we provided evidence for apparent selective cholinergic neurotoxicity. Exposure of 9-day-old cultures to 12.5 microM E...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334882
更新日期:1993-01-01 00:00:00
abstract::PICALM, a clathrin adaptor protein, plays important roles in clathrin-mediated endocytosis in all cell types. Recently, genome-wide association studies identified single nucleotide polymorphisms in PICALM gene as genetic risk factors for late-onset Alzheimer disease (LOAD). We analysed by western blotting with several...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1111-z
更新日期:2013-06-01 00:00:00
abstract::Gliomas induced in the rat by transplacental administration of ethylnitrosourea (ENU) are intensely immunoreactive for vimentin and scarcely for glial fibrillary acidic protein (GFAP). Since tumoral transformation takes place during the late fetal and early postnatal period, the sequential expression of the two glial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227665
更新日期:1992-01-01 00:00:00
abstract::This ultrastructural study deals with the synapses of primary dendrites of the anterior horn neurons in the lower lumbar spinal cords of seven patients with amyotrophic lateral sclerosis (ALS) who had mild neuronal depletion. Specimens from seven age-matched, neurologically normal individuals served as controls. In ea...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050426
更新日期:1996-01-01 00:00:00
abstract::With an incidence of approximately 350 in 100,000, stroke is the third leading cause of death and a major cause of disability in industrialized countries. At present, although progress has been made in understanding the molecular pathways that lead to ischemic cell death, the current clinical treatments remain poorly ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-009-0496-1
更新日期:2009-05-01 00:00:00
abstract::Focal cortical dysplasias (FCD) which represent a composite group of cortical malformations are increasingly recognized as morphological substrate for severe therapy-refractory epilepsy in children and young adults. However, presurgical evaluation remains challenging as not all FCD variants can be reliably detected by...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0920-1
更新日期:2012-02-01 00:00:00
abstract::Neurotrophins regulate the proliferation and differentiation of neurons in the central nervous system via a family of specialized receptors, including TrkA, TrkB, and TrkC. As little is known about their expression or potential role in human glial tissues and glial tumors, we undertook an immunohistochemical analysis ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050906
更新日期:1998-10-01 00:00:00
abstract::A case history illustrating the potential clinical significance of subependymoma is presented. Fine structural studies indicate that the tumor is composed of cells having the cytoplasmic features of ependyma, astrocytes, and transitional cells. Its composition and structure are alike those in the adult mammalian subep...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691968
更新日期:1977-11-28 00:00:00
abstract::Marinesco-Sjögren syndrome (MSS) features cerebellar ataxia, mental retardation, cataracts, and progressive vacuolar myopathy with peculiar myonuclear alterations. Most MSS patients carry homozygous or compound heterozygous SIL1 mutations. SIL1 is a nucleotide exchange factor for the endoplasmic reticulum resident cha...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1224-4
更新日期:2014-05-01 00:00:00
abstract::Isocitrate dehydrogenase 1 (IDH1) mutations are frequent in astrocytomas, oligoastrocytomas and oligodendrogliomas. We previously reported the generation of a mutation-specific antibody that specifically detects R132H mutated IDH1 protein (clone H09). Here, we investigate the feasibility of H09 immunohistochemistry to...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0770-2
更新日期:2011-02-01 00:00:00
abstract::Severe hypoxic-ischemic cerebral damage was produced in 8-day-old rats following permanent bilateral carotid artery occlusion and 15 min of ischemia. Cellular damage consisted of early necrosis and appearance of cells with apoptotic-like morphology (karyorrhectic cells) and cells with granular chromatin degeneration i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050753
更新日期:1997-12-01 00:00:00
abstract::This report deals with the expression of stress-response (heat-shock) protein 72 (srp 72) in a series of 95 primary human brain tumors and 21 carcinoma metastases to the central nervous system (CNS). Immunohistochemical procedures were employed; cells of the human cervical cancer line HeLa S3 were used as positive con...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227818
更新日期:1992-01-01 00:00:00
abstract::Immunofluorescence and immunogold labelling were used to localise the 43-kDa dystrophin-associated glycoprotein (43DAG) of the dystrophin-glycoprotein complex in control and Duchenne muscular dystrophy (DMD) biopsies. In control muscle 43DAG was localised by immunofluorescence to the periphery of the fibre and, by imm...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00313603
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
pub_type: 杂志文章
doi:10.1007/BF00687718
更新日期:1989-01-01 00:00:00
abstract::Apoptosis of neurons and glial cells has been shown to occur in the brain of patients with the acquired immune deficiency syndrome (AIDS) and was postulated as contributing to brain atrophy and white matter damage in these patients. Since apoptotic events may be induced by the Fas-Fas ligand (FasL) system, we analyzed...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051094
更新日期:1999-10-01 00:00:00
abstract::We investigated whether the brainstem is affected by the pathologic process of sporadic Creutzfeldt-Jakob disease (sCJD), with particular attention to brainstem atrophy, neuronal loss, pyramidal tract degeneration, and prion protein (PrP) deposition, in 33 patients with sCJD. Brainstem atrophy, particularly in the pon...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0981-0
更新日期:2005-06-01 00:00:00
abstract::The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of neuromuscular disorders subdivided into polymyositis (PM), sporadic inclusion body myositis (sIBM) and dermatomyositis (DM). Chemokines play an essential role in sustained inflammation associated with IIM. We studied the distribution of the alph...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0989-5
更新日期:2005-06-01 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
pub_type: 杂志文章
doi:10.1007/BF00690966
更新日期:1985-01-01 00:00:00
abstract::Pyroglutamylated amyloid-β (pE(3)-Aβ) has been suggested to play a major role in Alzheimer's disease (AD) pathogenesis as amyloid-β (Aβ) oligomers containing pE(3)-Aβ might initiate tau-dependent cytotoxicity. We aimed to further elucidate the associations among pE(3)-Aβ, full-length Aβ and hyperphosphorylated tau (HP...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1296-9
更新日期:2014-07-01 00:00:00
abstract::Impaired axonal transport of the fast or slow component has been reported in patients with sporadic amyotrophic lateral sclerosis (ALS), animal models for ALS, and familial ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) transgenic mice. However, little is known about the impairment of axonal transport in mutant S...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-1021-9
更新日期:2005-07-01 00:00:00
abstract::Ballooned neurons are histological features of several neurodegenerative diseases of the central nervous system. We describe the immunocytochemical staining of ballooned neurons in Pick's disease, unclassified dementia, corticonigral degeneration, pigment-spheroid degeneration and Alzheimer's disease. In all of these ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688042
更新日期:1986-01-01 00:00:00
abstract::Cyclooxygenases (COX, prostaglandin endoperoxide synthases, PGG/H synthases) are potent mediators of inflammation. While COX-1 is constitutively expressed in a wide range of tissues, COX-2 is cytokine inducible. Although COX-1 expression is observed in normal tissue, enhanced COX-2 expression has been attributed a key...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051075
更新日期:1999-09-01 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
pub_type: 杂志文章,评审
doi:10.1007/s004010050604
更新日期:1997-02-01 00:00:00
abstract::Defective interfering influenza virus particles lessened the yield of infective virus recovered from brain after intracerebral challange of 3-week old mice, but failed to affect the appearance of disease or lethality. In 7-week old mice, the presence of defective interfering influenza virus particles reduced both leth...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684666
更新日期:1976-03-15 00:00:00