Abstract：
:The post-mortem diagnosis of lysosomal storage diseases can be confounded by the unavailability of suitable material. Here we report the diagnosis of GM1-gangliosidosis in a cross-bred dog, from which only formalin-fixed brain was available, by a combination of electron microscopy and the detection of elevated levels of GM1-ganglioside within the tissue using the novel technique of electrospray ionisation tandem mass spectrometry. Electron microscopic examination of ultrathin sections of resin-embedded tissue revealed cytoplasmic inclusions (membranous cytoplasmic and zebra bodies) in brain stem and cerebellar neurons that were characteristic of a gangliosidosis. Glycolipids were extracted from the fixed tissue and analysed by tandem mass spectrometry. Two major ions were detected, which corresponded to GM1 (d18:1-C18:0) and Gm1 (d20:1-C18:0). Their identity was confirmed by comparison of their fragmentation patterns with those of authentic standards. The concentration of GM1 was approximately sixfold higher on a wet weight basis than in the brain of a normal control dog, confirming the diagnosis of GM1-gangliosidosis.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Whitfield P,Johnson AW,Dunn KA,Delauche AJ,Winchester BG,Franklin RJdoi
10.1007/s004010000187subject
Has Abstractpub_date
2000-10-01 00:00:00pages
409-14issue
4eissn
0001-6322issn
1432-0533journal_volume
100pub_type
杂志文章abstract：:We describe features of a patient that broadens the clinical and pathological spectrum of neurofilament inclusion disease (NFID). The patient was a 52-year-old man with a 5--6 year history of progressive, asymmetrical spastic weakness of the upper and lower extremities; L-DOPA-unresponsive parkinsonism; and SPECT evid...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0974-4
更新日期：2005-04-01 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：:Inflammatory processes have been implicated in the formation of senile plaques in the cerebral cortex of patients with dementia of the Alzheimer type (DAT), since several inflammation-induced proteins are present within these plaques. The relation between inflammatory components and other amyloid beta protein (A beta)...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050474
更新日期：1996-01-01 00:00:00
abstract：:A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691074
更新日期：1978-12-15 00:00:00
abstract：:Stereological estimates of mean volumes, surface areas, and cortical thicknesses were obtained on formalin-fixed brains from 19 men with AIDS and 19 controls. Volumes of neocortex, white matter, central brain nuclei, ventricles and archicortex were estimated using point counting and Cavalieri's unbiased principle for ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00334671
更新日期：1993-01-01 00:00:00
abstract：:A neuropathological study is presented of a case showing the association of tuberous sclerosis of the brain and dysplasia of the corpus callosum as well as omphalocele and malrotated colon. No signs of tuberous sclerosis were found in the internal organs. From a review of the literature this appears to be the fourth c...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF01273269
更新日期：1978-04-26 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：: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：:The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688176
更新日期：1989-01-01 00:00:00
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：:Low-grade neuroepithelial tumors (LGNTs) are diverse CNS tumors presenting in children and young adults, often with a history of epilepsy. While the genetic profiles of common LGNTs, such as the pilocytic astrocytoma and 'adult-type' diffuse gliomas, are largely established, those of uncommon LGNTs remain to be define...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-016-1539-z
更新日期：2016-06-01 00:00:00
abstract：:Pediatric ependymomas are highly recurrent tumors resistant to conventional chemotherapy. Telomerase, a ribonucleoprotein critical in permitting limitless replication, has been found to be critically important for the maintenance of tumor-initiating cells (TICs). These TICs are chemoresistant, repopulate the tumor fro...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-014-1327-6
更新日期：2014-12-01 00:00:00
abstract：:Histopathological, immunocytochemical, and electron microscopical investigations were carried out in a man with a protracted history of spastic paraparesis, adrenal insufficiency and hypogonadism. Pathological findings were identical with those of the few previously reported cases of adrenomyeloneuropathy (AMN) includ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690750
更新日期：1980-01-01 00:00:00
abstract：:SNAP-25 (a synaptosomal-associated protein of 25 kDa) has been shown to be involved both in synaptic vesicle exocytosis and in axonal outgrowth. In the present study, we investigated the changes in SNAP-25 immunoreactivity in the hippocampus of the Mongolian gerbil (Meriones unguiculatus) at different time points afte...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050795
更新日期：1998-03-01 00:00:00
abstract：:Amplification of the epidermal growth factor receptor (EGFR) gene is found in about 40% of glioblastomas (GBMs) but is rarely detected in GBM cell lines. We confirmed that the exceptional SKMG-3 GBM cell line retained amplified EGFR genes in vitro, and found that these sequences were concentrated on extra-chromosomal ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010000332
更新日期：2001-06-01 00:00:00
abstract：:Rats were given daily injections of 0.2 or 0.5 g/kg chlorophenoxyisobutyrate (clofibrate) for 9 to 46 days. Lower leg muscles were studied with light and electron microscopy. Daily treatment with 0.5 g/kg of the drug for 26--46 days caused myopathic changes. ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00703319
更新日期：1977-08-16 00:00:00
abstract：:Peroxiredoxin-ll (Prxll) and glutathione peroxidase-l (GPxl) are regulators of the redox system that is one of the most crucial supporting systems in neurons. This system is an antioxidant enzyme defense system and is synchronously linked to other important cell supporting systems. To clarify the common self-survival ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-005-1019-3
更新日期：2005-08-01 00:00:00
abstract：:The present study used cocultures of rat dorsal root ganglia (DRG) and peritoneal macrophages to define the role of activated complement components during demyelination. The complement cascade was activated in vitro by treatment of the cultures with natural rat serum and lipopolysaccharides. Complement activation was ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00318572
更新日期：1995-01-01 00:00:00
abstract：:Experimental neuropathy, characterized by endoneurial edema and demyelination, was induced by inoculating rabbits with a combination of Freund's complete adjuvant (FCA), gangliosides, lecithin and cholesterol. A less severe demyelinating neuropathy could be induced by treatment with FCA alone but no significant change...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00695496
更新日期：1987-01-01 00:00:00
abstract：:This report presents the ultrastructural nerve study of a patient with sensorimotor neuropathy by a shoe glue containing an association of n-hexane and methylethylketone. Giant axons, distended by microfilamentous proliferation typical of such cases of neuropathy, were found in significant amounts. An unusual histolog...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690990
更新日期：1981-01-01 00:00:00
abstract：:We studied a 27-year-old woman who died after a 6-year history of progressive dementia, dystonia, ataxia, apraxia, spasticity, choreoathetosis, visual and auditory hallucinations, and optic atrophy. Magnetic resonance imaging showed decreased intensity in the globus pallidus, substantia nigra, and dentate nuclei in T2...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010000224
更新日期：2000-11-01 00:00:00
abstract：:Virtually all phases of spinal cord injury pathogenesis, including inflammation, cell proliferation and differentiation, as well as tissue remodeling, are mediated in part by infiltrating monocyte-derived macrophages. It is now clear that these infiltrating macrophages have distinct functions from resident microglia a...
journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
doi：10.1007/s00401-019-01992-3
更新日期：2019-05-01 00:00:00
abstract：:A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：
更新日期：1976-01-01 00:00:00
abstract：:The cholinergic basal forebrain is divided into four subregions (Ch1-4), and cholinergic neuronal loss in the nucleus basalis of Meynert (Ch4) has been correlated with cognitive impairments in both Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). However, the Ch1-2 regions, which provide the major choline...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-005-0004-1
更新日期：2006-02-01 00:00:00
abstract：:This study was designed to compare the degree of reactive astrogliosis occurring around a puncture wound in the brain of normal rats and at different intervals after a similar puncture wound in rats with a portocaval anastomosis. The gliosis was evaluated by the number of astrocytes, the thickness of their processes a...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00310926
更新日期：1991-01-01 00:00:00
abstract：:Hydrocephalic neonates were observed in a small breeding colony of rats. Normal rats from this colony were obtained and brother-sister mated for seven generations. The overall prevalence of hydrocephalics was approximately 23%; however, in one subline, the prevalence approached 50%. Breeding data suggested the trait t...
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：:Using a fluorescent antibody technique, sections of the brains of patients with senile dementia or Alzheimer's disease containing senile plaques were treated with rabbit antihuman immunoglobulins labelled with FITC (fluorescein isothiocyanate, BBL). The senile plaques and the cerebral amyloid angiopathy (drusige Entar...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00689569
更新日期：1975-08-11 00:00:00
abstract：:Pilocytic astrocytomas are the most common astrocytic tumors of childhood and differ clinically and histopathologically from those astrocytomas that affect adults. Studies of adult astrocytic tumors have revealed allelic losses on chromosomes 10, 17p, 19q and alterations in the epidermal growth factor receptor (EGFR) ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00454903
更新日期：1993-01-01 00:00:00
abstract：:Isocitrate dehydrogenase 1 (IDH1) mutations are frequent in astrocytomas, oligoastrocytomas and oligodendrogliomas. We previously reported the generation of a mutation-specific antibody that specifically detects R132H mutated IDH1 protein (clone H09). Here, we investigate the feasibility of H09 immunohistochemistry to...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-010-0770-2
更新日期：2011-02-01 00:00:00
abstract：:The authors present the case of a hypertensive patient with a thrombosis of a saccular microaneurysm (SMA) and underlying cerebral (pontine) lacunae. This SMA lay at the junction between the main vessel and two small feeding arteries penetrating into the necrotic territory. Microhemorrhages were observed in and outsid...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688313
更新日期：1986-01-01 00:00:00
