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 ganglion cells in 11 sporadic ALS patients, and 12 age-matched, non-neurological control individuals in order to determine whether or not they are affected in ALS. In both the controls and ALS patients, unusual inclusion bodies were frequently observed in the mitochondria in the somata of the ganglion cells. The inclusions consisted of an aggregate of tubules measuring approximately 40 nm in diameter varying in size and number. Such inclusions were frequently present in the cristae and/or intermembrane space, often expanding to form large bundles in the dilated intermembrane space. These structures quite frequently protruded outward unilaterally or bilaterally and were partially surrounded by the outer membrane of the mitochondria. The number of inclusions was significantly higher in the ALS patients than in the controls (P < 0.0001). Regularly spaced transverse processes similar to the rungs of a ladder were occasionally observed in the intermembrane space, along with infrequent but markedly increased cristae and stubby mitochondria. We concluded that mitochondrial abnormalities may be involved in the degenerative processes in the dorsal root ganglion cells in sporadic ALS. These findings therefore suggest that ALS is a widespread, more generalized disorder than previously thought, and that the degeneration is not confined to the motor neuron system.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Sasaki S,Horie Y,Iwata Mdoi
10.1007/s00401-007-0299-1subject
Has Abstractpub_date
2007-12-01 00:00:00pages
633-9issue
6eissn
0001-6322issn
1432-0533journal_volume
114pub_type
杂志文章abstract::Bundles of paired helical filaments (PHF) accumulate in the pyramidal neurons that degenerate during Alzheimer's disease. This neurofibrillary degeneration is highly correlated with clinical signs of dementia. During the degenerating process, Tau proteins, which are the major antigenic components of PHF, are abnormall...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00308912
更新日期:1990-01-01 00:00:00
abstract::Surgical specimens of 4 intracranial saccular aneurysms were studied by scanning electron microscopy. The internal surface of the aneurysms showed crater-like defects and cytoplasmic bridges. In some areas the endothelium was preserved, but its longitudinal convolutions were higher and thicker than those found in unch...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691478
更新日期:1978-11-15 00:00:00
abstract::Immunohistochemical studies of monoamine neurons were performed to evaluate toxic effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) on young adult mice and compare them with those of their offspring. Mice, 9-11 weeks old (C57BL/6J), injected subcutaneously with a large dose of MPTP (17 mg/kg per day) duri...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294662
更新日期:1989-01-01 00:00:00
abstract::In a large series of 116 cortical resections for treatment of medically intractable epilepsy, 10 glial hamartomas and 11 neuronoglial malformative lesions were found. Glial hamartomas were astrocytic in 3 cases, oligodendrocytic in 6 and mixed oligoastrocytic in 1. Neuronoglial lesions corresponded to "focal cortical ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296190
更新日期:1994-01-01 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::Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to clinico-pathological overlap with other myopathies and the prevalence of TTN variants in control populations. Here, we present a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02257-0
更新日期:2021-01-15 00:00:00
abstract::Extracellular deposition of amyloid β-protein (Aβ) in amyloid plaques and intracellular accumulation of abnormally phosphorylated τ-protein (p-τ) in neurofibrillary tangles (NFTs) represent pathological hallmark lesions of Alzheimer's disease (AD). Both lesions develop in parallel in the human brain throughout the pre...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-019-02053-5
更新日期:2019-12-01 00:00:00
abstract::Equine grass sickness (EGS) is an acquired disease of unknown aetiology affecting horses kept at grass. The disease is characterised by postganglionic sympathetic and parasympathetic neuronal pathology and is categorised as a dysautonomia. This study undertook a systematic examination of brain stem cranial nerve nucle...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010000289
更新日期:2001-08-01 00:00:00
abstract::The brain tissue obtained from ninety-five cognitively unimpaired subjects, with ages ranging from 22 to 50 years upon death, were immunohistochemically assessed for neurodegenerative changes, i.e., hyperphosphorylated tau (HPτ) and β-amyloid (Aβ) pathology in predilection neuroanatomical areas. HPτ pathology was obse...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-011-0906-z
更新日期:2012-01-01 00:00:00
abstract::Experimental brain tumors were produced in rats by stereotactical implantation of various neoplastic cell lines (RG 2, RG1 2.2, G 13/11, F 98, RN 6, B 104, and E 367). Using autoradiographic, bioluminescence, and fluoroscopic methods, the following regional hemodynamic and metabolic parameters were measured on intact ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687050
更新日期:1986-01-01 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::Neuropathological changes were studied in a consecutive autopsy series of 135 cases, comprising 73% of all patients who died of AIDS in Switzerland between April 1981 and December 1987. Central nervous system involvement was found in 119 patients (88%), 19 of which had multiple concomitant intracerebral lesions. Among...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687372
更新日期:1989-01-01 00:00:00
abstract::A limited cortical resection including the rolandic fissure and the pre- and postcentral cortical regions was carried out in a patient suffering from epilepsia partialis continua resistant to antiepileptic drugs. The histological examination revealed several foci of very large neurons distributed with no laminar organ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00299415
更新日期:1992-01-01 00:00:00
abstract::In order to elucidate the immunohistochemical features of hydrocephalic ependyma, immunohistochemical examination was undertaken in 11 normal, postmortem brains (age range, 11 weeks' postconception to 6 months after birth) and 12 hydrocephalic brains (three cases each of congenital aqueductal stenosis, Dandy-Walker ma...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050493
更新日期:1996-07-01 00:00:00
abstract::To investigate the relationship between cerebral amyloid angiopathy and subcortical (lobar) hemorrhage, we examined the severity of amyloid deposition in the leptomeningeal, cortical and subcortical arteries in 28 autopsied elderly patients with cerebral amyloid angiopathy with subcortical hemorrhage, deep cerebral he...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00228574
更新日期:1993-01-01 00:00:00
abstract::We report two sporadic cases of tauopathy with unusual neuropathological features. The ages of the patients at death were 86 and 74 years, and the disease durations were 4 and 3 years, respectively. The former patient showed progressive dementia and amyotrophy (autopsy revealed that severe cervical spondylosis was res...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0093-5
更新日期:2006-09-01 00:00:00
abstract::Paired helical filaments (PHF) were isolated from autopsied brain of cases of Alzheimer dementia, and their polypeptides were identified with monoclonal antibodies to PHF by Western blots. The PHF polypeptide profile consisted of several bands with a size difference of less than 5 kilodalton (kDa) between adjacent ban...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00690830
更新日期:1985-01-01 00:00:00
abstract::Meningiomas are among the most frequent intracranial tumors. The secretory variant of meningioma is characterized by glandular differentiation, formation of intracellular lumina and pseudopsammoma bodies, expression of a distinct pattern of cytokeratins and clinically by pronounced perifocal brain edema. Here we descr...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-013-1093-x
更新日期:2013-03-01 00:00:00
abstract::In this immunocytochemical study, the brains of nine squirrel monkeys (Saimiri sciureus), ranging from 8 to 27 years of age, were examined for the presence and distribution of beta/A4 amyloid, a 4-kilodalton peptide. In aged squirrel monkeys, amyloid is associated primarily with intracerebral and meningeal capillaries...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00307691
更新日期:1990-01-01 00:00:00
abstract::The evolvement of amyloid beta (Abeta) deposition in the frontal cerebral cortex of 24 patients of increasing age with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D) was studied using end-specific monoclonal antibodies to Abetax-42 (Abeta42) or Abetax-40 (Abeta40) and markers for degenerating neu...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051143
更新日期:2000-04-01 00:00:00
abstract::We report an experimental model of Creutzfeldt-Jakob's disease (CJD) in mice leading to the formation of giant autophagic vacuoles (AV) in neurons of the cerebral cortex. These AV appear at the end of the incubation period (4-6 months postinoculation), together with spongy changes and clinical symptoms. Autophagy, a p...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688178
更新日期:1989-01-01 00:00:00
abstract::We estimated the total neurone number, glial number, and glial index (ratio glial cells/neurone) in the thalamic mediodorsal nucleus (MD) in seven patients suffering from Huntington's disease (HD; four males, three females, mean age 52.4 +/- 13.6 years) and age- and sex-matched controls (four males, three females, mea...
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::Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686206
更新日期:1988-01-01 00:00:00
abstract::Polyclonal antibodies directed towards synaptophysin were raised against a synthesised peptide corresponding to amino acids 246 to 260 of the human synaptophysin sequence. The antibodies, when applied for immunocytochemical staining, showed a staining pattern identical to that of the commercially available monoclonal ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00293325
更新日期:1994-01-01 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::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::Round granulated body (RGB) and eosinophilic hyaline droplets (EHDs) have been described as cytoplasmic inclusions of certain astrocytic tumors. In the previous literature, however, these inclusions have been described using various terms or regarded as nosologically the same entity. Light microscopically, RGB appeare...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296486
更新日期:1994-01-01 00:00:00
abstract::Neuronal intracytoplasmic inclusions were found in the ventrolateral and posterior nuclei of the thalamus of 3-month-old thiamine-deficient mice and quaking mice. An electron-microscopic study revealed that these inclusions were composed of condensation of parallel rectilinear 10-nm filaments with interconnecting cros...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691499
更新日期:1983-01-01 00:00:00
abstract::The ultrastructural features of SOD1-positive aggregates were determined to clarify whether these aggregates are associated with the pathogenesis of SOD1 mutant mice. We examined the spinal cord of transgenic mice expressing a G93A mutant human SOD1 gene with fewer copies (gene copy 10). At the early presymptomatic st...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0939-7
更新日期:2005-03-01 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