Abstract:
:Respiratory chain dysfunction leads to reactive oxygen species (ROS) generation with following oxidative stress and cellular damage. A histochemical and immunohistochemical study was performed on muscle biopsies from 17 patients with mitochondrial disease [chronic progressive external ophthalmoplegia (CPEO), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS), myoclonic epilepsy with ragged red fibers (MERRF)] to evaluate the expression pattern and location of manganese superoxide dismutase (MnSOD), copper-zinc superoxide dismutase (CuZnSOD) and reduced glutathione (GSH) in skeletal muscle fibers. Our data showed that: (1) MnSOD, CuZnSOD and GSH are expressed in fibers with respiratory chain deficiency; (2) the antioxidant induction is correlated with the degree of mitochondrial proliferation, but not with clinical phenotype, patients' age, duration of disease, biochemical defects or mitochondrial DNA abnormalities. In addition, we suggest that expression of MnSOD and GSH may be considered an initial, indirect sign of respiratory chain dysfunction because it is observed in the early stages of the disease.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Filosto M,Tonin P,Vattemi G,Spagnolo M,Rizzuto N,Tomelleri Gdoi
10.1007/s004010100455subject
Has Abstractpub_date
2002-03-01 00:00:00pages
215-20issue
3eissn
0001-6322issn
1432-0533journal_volume
103pub_type
杂志文章abstract::Nine dogs with canine distemper encephalitis (CDE) were examined with immunological techniques including demonstration of antibodies against canine distemper virus (CDV) in the serum and against myelin basic protein (MBP) in serum and in CSF. Mitogen stimulation tests of lymphocytes were also done. The brains were exa...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691174
更新日期:1982-01-01 00:00:00
abstract::An unusual tumor of the pituitary gland is reported. The most characteristic finding of the present tumor was the presence of numerous "colloid" and mucoid secretions. The cells were stellate in shape and there were intra- or intercellular lumina which were lined by many microvilli and occasional cilia. There were als...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687617
更新日期:1984-01-01 00:00:00
abstract::The central distal axonopathy induced in dogs by the administration of high doses of clioquinol is contrasted with the central-peripheral distal axonopathy precipitated by intoxication with 2,5-hexanedione. Mature, pure-bred Beagle dogs received a daily oral dose of 400 mg/kg of clioquinol for up to 7 months, or 1 ml ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690549
更新日期:1979-08-01 00:00:00
abstract::A case of cerebellar medullomyoblastoma in a young boy was investigated by electron microscopy. The neuroectodermal component shows the characteristics of a desmoplastic medulloblastoma. The mesodermal component consists of more or less differentiated cross-striated muscle cells. Undifferentiated muscle cells are very...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690521
更新日期:1979-12-01 00:00:00
abstract::The dynamics of cerebrospinal fluid (CSF) tau and Aβ biomarkers over time in Alzheimer's disease (AD) patients from prodromal pre-symptomatic to severe stages of dementia have not been clearly defined and recent studies, most of which are cross-sectional, present conflicting findings. To clarify this issue, we analyze...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1151-4
更新日期:2013-11-01 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::The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were e...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690463
更新日期:1984-01-01 00:00:00
abstract::Little information is available regarding the morphological changes in the mitochondria in amyotrophic lateral sclerosis (ALS). In particular, mitochondrial changes in dorsal root ganglion cells have not yet been examined. We therefore conducted an electron microscopic examination of the mitochondria in dorsal root ga...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0299-1
更新日期:2007-12-01 00:00:00
abstract::When 22 members of the BrainNet Europe (BNE) consortium assessed 31 cases with alpha-synuclein (alphaS) immunoreactive (IR) pathology applying the consensus protocol described by McKeith and colleagues in 2005, the inter-observer agreement was 80%, being lowest in the limbic category (73%). When applying the staging p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-009-0523-2
更新日期:2009-06-01 00:00:00
abstract::To assess whether amyloid plaque accumulation in the monkey brain can account for age-related cognitive impairment that begins at about 20 years of age, we measured plaque content in the brains of 14 rhesus monkeys aged 5-30 years. We used immunohistochemistry employing the monoclonal antibody 6E10, which is specific ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050735
更新日期:1997-11-01 00:00:00
abstract::The pathological changes in the nucleus basalis of Meynert (nbM) in 10 autopsied cases with Pick's disease were studied in comparison with 15 age-matched controls. Both the number and density of nerve cells and the degree of fibrillary gliosis were examined at the anterior, intermediate, and posterior divisions of the...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687402
更新日期:1989-01-01 00:00:00
abstract::A 77-year-old man suffered intermittent hemiconvulsions of unknown etiology on the left side for a period of about 5 weeks. At the autopsy, there was marked neuronal loss, severe proliferation of astrocytes and spongiform changes in the right cerebral cortex. The cerebral white matter showed loosening with astroglial ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687782
更新日期:1988-01-01 00:00:00
abstract::The deficiency of the cholinergic cortical projection system arising in the different basal forebrain structures collectively referred to as nucleus basalis of Meynert complex is a constant finding in Alzheimer's disease, a disorder which is neuro-pathologically characterised by the appearance of three intracerebral f...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690530
更新日期:1988-01-01 00:00:00
abstract::Solanezumab and Crenezumab are two humanized antibodies targeting Amyloid-β (Aβ) which are currently tested in multiple clinical trials for the prevention of Alzheimer's disease. However, there is a scientific discussion ongoing about the target engagement of these antibodies. Here, we report the immunohistochemical s...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1489-x
更新日期:2015-11-01 00:00:00
abstract::Healthy nodes of Ranvier are crucial for action potential propagation along myelinated axons, both in the central and in the peripheral nervous system. Surprisingly, the node of Ranvier has often been neglected when describing CNS disorders, with most pathologies classified simply as being due to neuronal defects in t...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-014-1305-z
更新日期:2014-08-01 00:00:00
abstract::A case of classic medulloblastoma that metastasized, despite the absence of local recurrence, to extraneural sites 7 years after treatment is reported. The metastases were, in contrast to the primary tumor, of large cell type and displayed abortive myogenic and, in one site, also rhabdoid differentiation. The primary ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-005-0986-8
更新日期:2005-05-01 00:00:00
abstract::Astrocytic alpha-synuclein-immunoreactive inclusions have recently been noted to develop in sporadic Parkinson's disease (PD). Here, the presence of immunoreactive astrocytes is reported in 14 autopsy cases with clinically diagnosed PD and a neuropathological stage of 4 or higher. The labeled astrocytes occur preferen...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0244-3
更新日期:2007-09-01 00:00:00
abstract::Frontotemporal dementia (FTD) is a prevalent neurodegenerative disease of heterogeneous histopathology. Neuropathological subtypes are identified on the basis of the presence or absence of tau- or ubiquitin-positive neuronal inclusions. Our recent work has established four disease stages that are independent of neurop...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0917-0
更新日期:2004-12-01 00:00:00
abstract::A quantitative study was performed on spinal cord lesions in seven patients with X-linked recessive spinal and bulbar muscular atrophy. The myelinated fiber density of the lateral corticospinal tracts at the T7 cord level was well preserved for both large and small myelinated fibers. On the other hand, neurons in the ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050575
更新日期:1997-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00687890
更新日期:1989-01-01 00:00:00
abstract::Experimental allergic neuritis was induced in Lewis rats by inoculation with autoreactive T cell lines sensitized to residue 57-81 of P2 myelin protein. Control rats received cells derived from immunization to complete Freund's adjuvant alone. Endoneurial fluid pressure (EFP) was measured in both sciatic nerves at 0, ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296547
更新日期:1991-01-01 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
pub_type: 杂志文章
doi:10.1007/s00401-004-0880-9
更新日期:2004-09-01 00:00:00
abstract::We described a new type of cytoplasmic inclusion in the choroidal epithelial cells of humans. The inclusions usually appeared as brown, round or elongated bodies with or without an inner core, ranging in size from 1.3 to 7.0 micron. Histochemically, they contained polysaccharides, proteins and compound lipids. Ultrast...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687675
更新日期:1988-01-01 00:00:00
abstract::Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease associated with repetitive mild impact traumatic brain injury from contact sports. Recently, a consensus panel defined the pathognomonic lesion for CTE as accumulations of abnormally hyperphosphorylated tau (p-tau) in neurons (neurofibrillary tangle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-016-1649-7
更新日期:2017-03-01 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::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::Modern neuropathology serves a key function in the multidisciplinary management of brain tumor patients. Owing to the recent advancements in molecular neurooncology, the neuropathological assessment of brain tumors is no longer restricted to provide information on a tumor's histological type and malignancy grade, but ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0736-4
更新日期:2010-11-01 00:00:00
abstract::Numbers of synapses were counted in columns of neuropil orientated at right angles to the pia in frontal and temporal regions from postmortem brains. In the frontal region of 28 patients aged 26-90 years there were 1.2 X 10(8) mm3 (SD 0.03 X 10(8], and in the temporal of 18 patients aged 16-90 years there were 1.4 X 1...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00684929
更新日期:1983-01-01 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
pub_type: 杂志文章
doi:10.1007/BF00389504
更新日期:1994-01-01 00:00:00
abstract::Two spontaneous neurodegenerative diseases of the horse, equine motor neuron disease (EMND) and equine degenerative myeloencephalopathy (EDM), have been associated with alpha-tocopherol deficiency, and both were characterized by prominent accumulations of endothelial lipopigment in the small vessels of the spinal cord...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296510
更新日期:1995-01-01 00:00:00