Abstract:
:The intracellular localization of myoglobin(Mb) mRNA in the skeletal muscles of normal subjects and patients with Duchenne muscular dystrophy(DMD) or amyotrophic lateral sclerosis(ALS) was examined by in situ hybridization using a biotin-labeled cDNA probe. In cross sections of normal muscles, Mb mRNA signals were demonstrated to be diffusely distributed as granular reaction products throughout the sarcoplasm, and in longitudinal sections the products were observed preferentially on the A-band. In DMD or ALS muscles, the distribution of granular mRNA signals showed some similarities with that in normal muscles, although degenerated fibers revealed a heterogenous distribution of the signals. In DMD muscles, the optical density(OD) of stained signal was higher in non-atrophic fibers and lower in atrophic fibers than in normal muscles. In ALS muscles, the OD was lower than in normal muscles. These results suggest that Mb mRNA is distributed preferentially on the A-band of the muscle fibers, and that in diseased muscle fibers Mb synthesis is affected by pathological changes.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Mitsui T,Kawai H,Naruo T,Nishino H,Saito Sdoi
10.1007/BF00369442subject
Has Abstractpub_date
1993-01-01 00:00:00pages
313-8issue
4eissn
0001-6322issn
1432-0533journal_volume
86pub_type
杂志文章abstract::Massive GGGGCC repeat expansion in the first intron of the gene C9orf72 is the most common known cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Despite its intronic localization and lack of an ATG start codon, the repeat region is translated in all three reading fra...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1189-3
更新日期:2013-12-01 00:00:00
abstract::Although it is known that deletions or mutations of the SMN1 gene on chromosome 5 cause decreased levels of the SMN protein in subjects with proximal autosomal recessive spinal muscular atrophy (SMA), the exact sequence of pathological events leading to selective motoneuron cell death is not fully understood yet. In t...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-008-0411-1
更新日期:2008-09-01 00:00:00
abstract::Neurotropic herpesviruses can establish lifelong infection in humans and contribute to severe diseases including encephalitis and neurodegeneration. However, the mechanisms through which the brain's immune system recognizes and controls viral infections propagating across synaptically linked neuronal circuits have rem...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1885-0
更新日期:2018-09-01 00:00:00
abstract::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 magnesi...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688211
更新日期:1989-01-01 00:00:00
abstract::Cystic necrosis in the cerebellar white matter was found in three premature infants. The necrosis was characteristically localized in the center of the white matter of the superficial cerebellar folia, sparing the overlying cortex. The patients were aged between 28 and 34 gestational weeks, and had a clinical history ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00315013
更新日期:1995-01-01 00:00:00
abstract::Since we reported a case of acute relaxant-steroid myopathy (ARSM) in 1994, we continued histological studies and compared the findings with those in a case of corticosteroid myopathy (CM). It was revealed that (1) dystrophin, spectrin, beta dystroglycan, and sarcoglycans on the cell surface were decreased, (2) regula...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051022
更新日期:1999-05-01 00:00:00
abstract::Protein aggregation is a major pathological hallmark of many neurodegenerative disorders including polyglutamine diseases. Aggregation of the mutated form of the disease protein ataxin-3 into neuronal nuclear inclusions is well described in the polyglutamine disorder spinocerebellar ataxia type 3 (SCA3 or Machado-Jose...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-010-0717-7
更新日期:2010-10-01 00:00:00
abstract::Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by an expanded trinucleotide CAG repeat in the gene coding for huntingtin. Deregulation of chromatin remodeling is linked to the pathogenesis of HD but the mechanism remains elusive. To identify what genes are deregulated by trimethyla...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1103-z
更新日期:2013-05-01 00:00:00
abstract::Glioblastomas, the most malignant human brain tumors, are characterized by marked aneuploidy, suggesting chromosomal instability which may be caused by a defective mitotic spindle checkpoint. We screened 22 glioblastomas for mutations in the mitotic spindle check-point genes hBUB1, hBUBR1 and hBUB3. DNA sequencing rev...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100366
更新日期:2001-04-01 00:00:00
abstract::This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308960
更新日期:1989-01-01 00:00:00
abstract::This study was undertaken to elucidate morphological changes in the synaptic area of the Purkinje cell dendritic spines when granule cells were decreased in number. The mice were injected s.c. with 30 mg/kg b.w. of cytosine arabinoside on days 2, 3, and 4, and on days 7, 8 and 9, and were designated as group I and gro...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685343
更新日期:1983-01-01 00:00:00
abstract::Alpha-synuclein is known to play an important role in several neurodegenerative diseases. Moreover, it is expressed in central nervous system neuronal tumors, and another member of the synuclein family, gamma-synuclein, is overexpressed in breast and ovarian carcinomas. However, the expression of alpha-synuclein has n...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010100443
更新日期:2002-02-01 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
pub_type: 杂志文章
doi:10.1007/s00401-007-0198-5
更新日期:2007-04-01 00:00:00
abstract::Two cases out of a sample of 41 fetuses and infants with prosencephalic malformation, observed at the Institute of Pathology and Department of Neuropathology of the University of Heidelberg, are described here in detail. These cases presented grossly with microcephaly and missing forebrain, appearing to be cases of ap...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007438
更新日期:2000-03-01 00:00:00
abstract::Research efforts during the last decade have deciphered the basic molecular mechanisms governing mitochondrial fusion and fission. We now know that in mammalian cells mitochondrial fission is mediated by the large GTPase dynamin-related protein 1 (Drp1) acting in concert with outer mitochondrial membrane (OMM) protein...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-011-0930-z
更新日期:2012-02-01 00:00:00
abstract::Alterations in sphingolipid metabolism are described to contribute to various neurological disorders. We here determined the expression of enzymes involved in the sphingomyelin cycle and their products in postmortem brain tissue of multiple sclerosis (MS) patients. In parallel, we investigated the effect of the sphing...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-012-1014-4
更新日期:2012-09-01 00:00:00
abstract::The expression of members of the Jun family of transcription factors was examined by immunohistochemistry, Western blotting, in situ hybridization and Northern blotting in the developing and adult rat brain following colchicine administration. Apoptotic cells, as revealed by their typical morphology and positive stain...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051059
更新日期:1999-08-01 00:00:00
abstract::Using a photochemical method silver was demonstrated in the brains of 1-, 14-, and 45-day-old rats which had been exposed to silver on gestational days 18 and 19. In the brain tissue of new-born rats, silver was found in the lysosomes of neurons and astroglia. Minor changes in the anatomic distribution of silver were ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691995
更新日期:1983-01-01 00:00:00
abstract::Damage to axons in acute multiple sclerosis (MS) lesions is now well established but the mechanisms of this damage remain obscure. Here we have applied a panel of antibodies that identify cell populations and proteins contained in them with a view to detecting those cells and proteins that are localised particularly c...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0045-0
更新日期:2006-04-01 00:00:00
abstract::A 79-year-old woman had a 10-year history of dementia, initially presenting as non-fluent aphasia. Magnetic resonance imaging showed frontal atrophy (left greater than right) and hyperintense foci within white matter. Neuropathologically, there was severe frontal atrophy due to cortical neuronal loss with spongy chang...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0719-9
更新日期:2003-08-01 00:00:00
abstract::In adult mice, administration of the anticonvulsive drug phenytoin caused focal swellings along the Purkinje cell axon correlated with ataxia and incoordination of movements. In our model, we used murine cerebellar slice cultures to study the influence of phenytoin on postnatal Purkinje cell axon differentiation. Almo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050844
更新日期:1998-06-01 00:00:00
abstract::APO2 ligand (APO2L)/TRAIL is a novel member of the tumor necrosis factor cytokine family and a potent inducer of apoptosis in tumor cell lines. We recently reported that APO2L is consistently expressed in low-grade astrocytomas, anaplastic astrocytomas, glioblastomas, and cell lines derived thereof, and that malignant...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/pl00007399
更新日期:2000-01-01 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
pub_type: 杂志文章
doi:10.1007/s00401-010-0659-0
更新日期:2010-04-01 00:00:00
abstract::We examined the immunohistochemical localization of p35nck5a, the regulatory subunit of cyclin-dependent kinase 5 (cdk5), in brains obtained postmortem from patients with Parkinson's disease (PD) and controls. We found p35nck5a immunoreactivity in Lewy bodies (LB) in the substantia nigra, locus ceruleus, and neocortex...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050687
更新日期:1997-08-01 00:00:00
abstract::We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the e...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-002-0543-7
更新日期:2002-09-01 00:00:00
abstract::In order to further evaluate the parameters whereby intracerebral administration of recombinant α-synuclein (αS) induces pathological phenotypes in mice, we conducted a series of studies where αS fibrils were injected into the brains of M83 (A53T) and M47 (E46K) αS transgenic (Tg) mice, and non-transgenic (nTg) mice. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1268-0
更新日期:2014-05-01 00:00:00
abstract::The peripheral immune system plays a critical role in aging and in the response to brain injury. Emerging data suggest inflammatory responses are exacerbated in older animals following ischemic stroke; however, our understanding of these age-related changes is poor. In this work, we demonstrate marked differences in t...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-018-1859-2
更新日期:2018-07-01 00:00:00
abstract::Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a neurodegenerative disorder characterized by diabetes mellitus and optic atrophy as well as diabetes insipidus and deafness in many cases. We report the post-mortem neuropathologic findings of a patient with Wolfram syn...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0546-8
更新日期:2009-09-01 00:00:00
abstract::Sensitive detection of alpha-synuclein (alpha-syn) pathology is important in the diagnosis of disorders like Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy and in providing better insights into the etiology of these diseases. Several monoclonal antibodies that selectively react with aggreg...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-008-0375-1
更新日期:2008-07-01 00:00:00
abstract::To investigate the pathogenetic role of human T lymphocyte virus type I (HTLV-I) in central nervous system disease, a rat model for HTLV-I-associated myelopathy/tropical spastic paraparesis, designated as HAM rat disease, has been established. Wistar-King-Aptekman-Hokudai strain rats with induced HTLV-I infection deve...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050962
更新日期:1999-02-01 00:00:00