Abstract：
:Previous investigators have suggested that proteolysis by calpain, a Ca2+-dependent protease, causes muscle fiber degradation in Duchenne and Becker muscular dystrophies (DMD/BMD). Recent evidence indicates that the nonlysosomal ATP-ubiquitin-dependent proteolytic complex (proteasomes) participates in muscle wasting during various catabolic states and in muscle fiber degradation in physiological or pathological conditions. To elucidate the possible role of proteasomes in dystrophic muscles, routine histochemistry and immunohistochemistry of 26S proteasomes were performed on muscle biopsy specimens obtained from patients with various neuromuscular disorders including DMD/BMD, polymyositis (PM), amyotrophic lateral sclerosis, and peripheral neuropathies, and on normal human muscle specimens. Immunohistochemically, proteasomes were located in the cytoplasm in normal human muscle, but their staining intensity was faint. Compared to control muscles, abnormal increases in both proteasomes and ubiquitin were demonstrated mainly in the cytoplasm of necrotic fibers and to a lesser extent in regenerative fibers in DMD/BMD and PM. Non-necrotic, atrophic fibers in all diseased muscles showed moderate or weak immunoreactions for the proteins; their staining intensities were stronger than those of control muscle fibers. Both proteins often colocalized well. Not all dystrophin-deficient muscle fibers showed a strong reaction for proteasomes. Our results showed increased proteasomes in necrotic and regenerative muscle fibers in DMD/ PMD, although this may not be disease-specific up-regulation. We suggest that the ATP-ubiquitin-dependent proteolytic pathway as well as the nonlysosomal calpain pathway may participate in muscle fiber degradation in muscular dystrophy.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Kumamoto T,Fujimoto S,Ito T,Horinouchi H,Ueyama H,Tsuda Tdoi
10.1007/s004010000229subject
Has Abstractpub_date
2000-12-01 00:00:00pages
595-602issue
6eissn
0001-6322issn
1432-0533journal_volume
100pub_type
杂志文章abstract：:The long-term survival of oligodendrocytes in the absence of axons in adult animals was studied following Wallerian degeneration of the optic nerves of adult rats for periods up to 22 months. In contrast to the findings in development and in young animals, large numbers of oligodendrocytes survived during this time pe...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308922
更新日期：1990-01-01 00:00:00
abstract：:Oxidative stress has been implicated in the pathogenesis of a number of diseases including Alzheimer's disease (AD). The oxidative stress hypothesis of AD pathogenesis, in part, is based on beta-amyloid peptide (Abeta)-induced oxidative stress in both in vitro and in vivo studies. Oxidative modification of the protein...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-009-0517-0
更新日期：2009-07-01 00:00:00
abstract：:Exercise intolerance associated with myalgias, muscle cramps or myoglobinuria may be associated with a dystrophinopathy. A search for abnormal dystrophin expression (using immunohistochemistry, immunoblot and DNA analysis) was carried out in a series of 15 patients. They were selected because they presented exercise i...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050671
更新日期：1997-07-01 00:00:00
abstract：:A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690395
更新日期：1984-01-01 00:00:00
abstract：:Human adenovirus type 12 (Ad 12) was inoculated intraperitoneally, intrapleurally, intramuscularly or subcutaneously into newborn rodents. Tumors developed preferentially in the peritoneal cavities in 93.9% of the hamsters and 82.6% of the mice, but none in rats; in contrast to the high incidence of brain tumors in ra...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687575
更新日期：1989-01-01 00:00:00
abstract：:The selective toxicity of silica dust for macrophages has been used to assess the role of these cells in experimental allergic neuritis (EAN). Inbred Lewis rats were inoculated with bovine dorsal roots in Freund's complete adjuvant (day 0). In two experiments, animals received 200 mg of silica dust in 1 cm3 of saline ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687614
更新日期：1984-01-01 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：:Oral administration of N-[4-(3-ethoxy-2-hydropropoxy)phenyl] acrylamide (EHA) induced selective granule cell destruction in the granular layer of the cerebellar cortex together with neurological signs, such as delayed righting reflex, gait or truncal ataxia, and convulsion. Neuropathologically, it caused multifocal gr...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051133
更新日期：2000-04-01 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：:Cryostat sections of 12 gliomas and of 3 peritumoral brain tissue samples were investigated for mononuclear cell infiltration by immunohistochemistry, concentrating on cells expressing monocyte/macrophage markers. Only low numbers of T cells were detected in the tumors, whereas in average 20%-30% of all cells present ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294647
更新日期：1990-01-01 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：:Although much work has been directed recently towards unravelling the protein chemistry of neurofibrillary tangle (NFT) and senile plaque (SP) components in Alzheimer's disease, the pathogeneses of these lesions remains largely unknown and the problem of their relationship is unresolved. In particular, although paired...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687379
更新日期：1989-01-01 00:00:00
abstract：:The ultrastructure of the nervous tissue in a benign ovarian teratoma is described. This tissue was organized into areas having both "meningeal" and "ependymal" surfaces, between which were found astrocytes, ependymal cells, neurones with synapses and microglia. These cells all had ultrastructural similarities to thei...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00686623
更新日期：1987-01-01 00:00:00
abstract：:Widespread damage to axons in the white matter of the brain is a well-recognised consequence of non-missile head injury. This diffuse axonal injury is characterised by a gradual swelling of the axon associated with an accumulation of cellular organelles and proteins. We have investigated the relationship between the s...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010051069
更新日期：1999-08-01 00:00:00
abstract：:Abundant proliferation of cells having the histologic and tinctorial features of microglia, were seen in the brain of 34-year old man who suffered cardiac arrest 10 days before death and whose bone marrow was nearly totally depleted of white-blood-cell precursors. It is suggested, that in adult human brains there are ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691588
更新日期：1978-09-15 00:00:00
abstract：:Using a 3H-labelled virion DNA probe applied to tissue sections, we have previously identified the precise microscopic anatomical location of herpes simplex virus (HSV) during the acute and latent stages of infection of the mouse trigeminal ganglia and central nervous system (CNS). In the present investigation, we com...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00692842
更新日期：1987-01-01 00:00:00
abstract：:The effects of ageing, cachexia and neoplasms on striated muscle were examined in histological sections of an autopsy material. Paraffin sections were examined from four separate muscles of 30 previously healthy subjects who died suddenly, from eight cases with cachexia and from 16 cases with cachexia and neoplasms. I...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00685374
更新日期：1982-01-01 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
pub_type： 杂志文章
doi：
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
pub_type： 杂志文章
doi：10.1007/BF00687824
更新日期：1985-01-01 00:00:00
abstract：:Three cases of spontaneous olfactory neuroblastoma (ONB) in domestic cats were morphologically and immunocytochemically characterized. Diagnostic light microscopic features included Flexner and Homer-Wright rosettes, while ultrastructurally the cells had neuritic processes, intracellular intermediate filaments, and in...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294617
更新日期：1990-01-01 00:00:00
abstract：:Focal cortical dysplasia (FCD) is a localized malformation of cortical development and is the commonest cause of severe childhood epilepsy in surgical practice. Children with FCD are severely disabled by their epilepsy, presenting with frequent seizures early in life. The commonest form of FCD in children is character...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-013-1135-4
更新日期：2013-08-01 00:00:00
abstract：:In this study, we used immunohistochemistry to screen for alpha-synuclein pathology in the brains of 241 individuals without clinical evidence of neurologic disease, and discovered 36 cases (15%) with incidental Lewy bodies (LBs) and one case, a 96-year-old woman (0.4%), with inclusions similar to those seen in multip...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-008-0398-7
更新日期：2008-09-01 00:00:00
abstract：:Brain tumors are the most common solid tumors of childhood, and the genetic drivers and optimal therapeutic strategies for many of the different subtypes remain unknown. Here, we identify that bithalamic gliomas harbor frequent mutations in the EGFR oncogene, only rare histone H3 mutation (in contrast to their unilate...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-020-02155-5
更新日期：2020-06-01 00:00:00
abstract：:Gliomatosis cerebri (GC), a rare and deadly CNS neoplasm characterized by involvement of at least three cerebral lobes, predominantly affects adults. While a few small series have reported its occurrence in children, little is known about the molecular characteristics of pediatric GC. We reviewed clinical, radiologica...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-015-1532-y
更新日期：2016-02-01 00:00:00
abstract：:Among the various mechanisms proposed to explain the pathogenesis of cerebral lesions in human immunodeficiency virus (HIV)-induced encephalitis, a cytokine-mediated action has found most favour. Indeed, elevated expression of cytokines such as interleukin (IL)-1 and tumor necrosis factor-alpha (TNF-alpha), thought to...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050457
更新日期：1996-01-01 00:00:00
abstract：:The suprascapular nerve from 14 horses, which had no clinical evidence of spinatus muscle atrophy, were obtained to determine whether the nerve was sub-clinically compressed at the scapular edge. The nerves were divided into three portions, proximal and distal to the scapular edge and as it reflected around it. In nin...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688338
更新日期：1987-01-01 00:00:00
abstract：:This report concerns an immunocytochemical and ultrastructural study of the motor cortices of 11 patients with amyotrophic lateral sclerosis (ALS). Specimens from 12 normal individuals served as controls. Antibodies against phosphorylated neurofilament (PNF; 200 kDa), ubiquitin, glial fibrillary acidic protein (GFAP) ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00293318
更新日期：1994-01-01 00:00:00
abstract：:Defects of major histocompatibility complex (MHC) class I antigen-processing machinery (APM) components have been shown to contribute to immune escape of malignant cells. We investigated the expression of APM components in astrocytomas without detectable defects in HLA class I antigen expression and correlated it with...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-007-0231-8
更新日期：2007-08-01 00:00:00
abstract：:A case of multifocal axonopathy associated with intrathecal methotrexate (IT MTX) and radiation therapy is presented. A 33-year-old woman suffering from meningeal carcinomatosis of breast cancer origin had developed prominent multifocal axonal degeneration in the cerebral white matter after treatment with IT MTX thera...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294671
更新日期：1989-01-01 00:00:00
abstract：:The incidence of CNS lymphoma has increased significantly in the past 30 years, primarily in the elderly and immunocompromised. While T-cell lymphomas comprise 15-20% of systemic lymphomas, they comprise less than 4% of primary CNS lymphomas, suggesting that they may be under-recognized compared to their systemic coun...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-007-0338-y
更新日期：2008-03-01 00:00:00
