Abstract:
:There is evidence that apoptotic cell death mechanisms contribute to muscle fibre loss in dystrophinopathies, but little knowledge about the activators of the final degrading caspase cascade in muscle fibre apoptosis. As mitochondria-related activation of this caspase cascade, through e.g. APAF-1, could not be proven in dystrophin-deficient muscle, this study searches for other prospective candidates that may directly trigger apoptotic cell degradation by mitochondria-independent pathways involving the interaction of tumour necrosis factor-alpha (TNF-alpha) and TRAIL with death receptors and subsequent activation of caspase-8. The expression of TNF-alpha, TNF-R1, TRAIL, NF-(kappa)B and caspase-8 were studied in muscle biopsy specimens from 14 patients with a dystrophinopathy [10 Duchenne muscular dystrophies (DMD), 2 Becker MD, and 2 DMD carriers] by immunohistochemistry and Western blotting. In all types of dystrophinopathies, necrotic muscle fibres undergoing myophagocytosis displayed strong expression of TNF-alpha, TNF-R1, and TRAIL, which, however, was attributed to phagocytosing cells and not to the muscle fibres themselves. There was no up-regulation in normal-shaped or atrophic non-necrotic muscle fibres, or in intact muscle fibre segments adjacent to segmental necrosis and myophagocytosis. The expression profiles of caspase-8 and NF-(kappa)B resembled that of normal control muscle. There were likewise no significant differences in the Western blot analyses between normal control and dystrophin-deficient muscle. Based on these findings, a contribution of TNF-alpha or TRAIL-mediated cell death pathways to muscle fibre apoptosis or necrosis in dystrophinopathies could not be confirmed.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Tews DSdoi
10.1007/s00401-004-0934-zsubject
Has Abstractpub_date
2005-02-01 00:00:00pages
217-25issue
2eissn
0001-6322issn
1432-0533journal_volume
109pub_type
杂志文章abstract::Aberrant peripheral nerve fiber bundles, i.e., masses of peripheral nerve fibers without perineural sheath cells, are found in the perivascular spaces of some spinal cords. The 514 spinal cords examined for these structures were separated into two groups; 507 spinal cords without definite necrotic lesions (group 1) an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308951
更新日期:1989-01-01 00:00:00
abstract::The morphological effects of two chemically different neuroactive drugs (chlorpromazine and phenobarbital) on vasculogenesis in rat cerebellum were examined to determine the presence of vascular alterations. Therapeutic dosages of both drugs were chronically administered to separate groups of maternal rats beginning o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692188
更新日期:1982-01-01 00:00:00
abstract::To clarify the role of prostaglandins in peripheral nerve demyelination, we examined the expression of cyclooxygenase-2 (COX-2) using selected nerve specimens from patients with chronic inflammatory demyelinating polyneuropathy. COX-2 protein was up-regulated in macrophages causing active demyelination. In situ hybrid...
journal_title:Acta neuropathologica
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更新日期:2001-02-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
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更新日期:2006-03-01 00:00:00
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journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
pub_type: 杂志文章
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更新日期:1990-01-01 00:00:00
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journal_title:Acta neuropathologica
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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
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doi:10.1007/BF00686623
更新日期:1987-01-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-014-1342-7
更新日期:2014-12-01 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
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更新日期:2005-07-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688050
更新日期:1986-01-01 00:00:00
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journal_title:Acta neuropathologica
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更新日期:2009-09-01 00:00:00
abstract::Axonal degeneration contributes to the transient and permanent neurological deficits seen in multiple sclerosis, an inflammatory disease of the central nervous system. To study the immunological mechanisms causing axonal degeneration, we induced experimental autoimmune encephalomyelitis (EAE) in wildtype Lewis rats an...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0047-y
更新日期:2006-06-01 00:00:00
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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::Spinal cord of the rat was investigated immunohistochemically to detect signs of extravasation of fibronectin in animals in which the cord was subjected to different degrees of compression trauma. Immunohistochemistry was performed after survival periods of 4 and 24 h and parallel sections were incubated for albumin i...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00227738
更新日期:1992-01-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691790
更新日期:1979-10-01 00:00:00
abstract::Human immune deficiency virus (HIV) disease may be associated, neuropathologically, with significant neuronal loss and clinically with a severe dementia. However, the significance of neuronal loss in the development of dementia has not been established. In this study we have undertaken a stereological determination of...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296490
更新日期:1994-01-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686877
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更新日期:2006-07-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687744
更新日期:1981-01-01 00:00:00
abstract::Neuropathological studies have revealed that the brains of HIV-1-infected AIDS patients show the typical encephalitis and, in addition, neuronal loss. More recently, this neuronal cell loss has been thought to take place via programmed cell death (apoptosis) which has been demonstrated by an in situ end labelling (ISE...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050409
更新日期:1996-01-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051037
更新日期:1999-06-01 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
pub_type: 杂志文章
doi:10.1007/BF00294242
更新日期:1990-01-01 00:00:00
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 d...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000229
更新日期:2000-12-01 00:00:00
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journal_title:Acta neuropathologica
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doi:10.1007/s00401-002-0543-7
更新日期:2002-09-01 00:00:00
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685010
更新日期:1978-08-07 00:00:00
abstract::We have developed a functional vegetative model by an 18-min clamping of the ascending aorta combined with a bypass formation between the aorta to right atrium and the aorta to femoral vein. Complete global brain ischemia (CGBI) induced for 18 min with this model provided the following distinct advantages: cardiopulmo...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294614
更新日期:1990-01-01 00:00:00