Abstract：
:Pelizaeus-Merzbacher disease (PMD) is an untreatable and fatal leukodystrophy. In a model of PMD with perturbed blood-brain barrier integrity, cholesterol supplementation promotes myelin membrane growth. Here, we show that in contrast to the mouse model, dietary cholesterol in two PMD patients did not lead to a major advancement of hypomyelination, potentially because the intact blood-brain barrier precludes its entry into the CNS. We therefore turned to a PMD mouse model with preserved blood-brain barrier integrity and show that a high-fat/low-carbohydrate ketogenic diet restored oligodendrocyte integrity and increased CNS myelination. This dietary intervention also ameliorated axonal degeneration and normalized motor functions. Moreover, in a paradigm of adult remyelination, ketogenic diet facilitated repair and attenuated axon damage. We suggest that a therapy with lipids such as ketone bodies, that readily enter the brain, can circumvent the requirement of a disrupted blood-brain barrier in the treatment of myelin disease.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Stumpf SK,Berghoff SA,Trevisiol A,Spieth L,Düking T,Schneider LV,Schlaphoff L,Dreha-Kulaczewski S,Bley A,Burfeind D,Kusch K,Mitkovski M,Ruhwedel T,Guder P,Röhse H,Denecke J,Gärtner J,Möbius W,Nave KA,Saher Gdoi
10.1007/s00401-019-01985-2subject
Has Abstractpub_date
2019-07-01 00:00:00pages
147-161issue
1eissn
0001-6322issn
1432-0533pii
10.1007/s00401-019-01985-2journal_volume
138pub_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：:An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its int...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050805
更新日期：1998-03-01 00:00:00
abstract：:Clinical and morphological findings in an 8-year-old Jewish girl with spongy leukodystrophy are presented. Ultrastructural changes indicated that this may be a form of Canavan's disease with some atypical features. Biochemical measurements indicated that synaptosomal membranes prepared from the striatum but not from t...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691321
更新日期：1981-01-01 00:00:00
abstract：:Wistar rats were given intravenous (i.v.) horseradish peroxidase (HRP) in saline in doses commonly used to study vascular permeability and the blood-brain barrier. Samples of blood were taken from conscious animals via indwelling catheters at intervals up to 6 h after the HRP injection. Plasma concentrations of adreno...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687945
更新日期：1986-01-01 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：:We examined the effect of lactic acid on cultured human glioma cell lines expressing glial fibrillary acidic protein (GFAP), vimentin and neuron-specific enolase (NSE). The growth of the cells was inhibited by the lactic acid in a dose-dependent manner. At 56 mM of lactic acid, the surviving cells of the KNS-42-c2 cel...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00296110
更新日期：1990-01-01 00:00:00
abstract：:The present investigation examined the morphological characteristics of epiplexus macrophages following a single intracisternal injection of the antigen, bacillus Calmette-Guerin (BCG). Three days following injection of BCG (0.5 - 4.0 X 10(8) viable microorganisms), mongrel dogs were perfused with buffered aldehydes. ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00690545
更新日期：1979-08-01 00:00:00
abstract：:A morphometric study of the basal nucleus of Meynert (bnM) has been performed in a 70-year-old man with a 4-year history of pathologically confirmed progressive supranuclear palsy (PSP). An important neuronal loss (52%) was demonstrated in the bnM. This finding has not been previously documented with morphometric meth...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00697397
更新日期：1983-01-01 00:00:00
abstract：:Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to D. The strength of this histopathologic classification lies in the association between FTLD-TDP subtypes and various clinical and genetic f...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-017-1679-9
更新日期：2017-07-01 00:00:00
abstract：:A description is given of three cases of ganglioneuromas, which originated from ganglia of the sympathetic chain of hamsters. Other reports of such tumors in this species are reviewed and the literature relative to hamster ganglioneuromas presented. In addition, the possible genesis of origin from adult ganglion cells...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00696794
更新日期：1975-10-01 00:00:00
abstract：:Glioblastomas, the most malignant human brain tumors, are characterized by marked aneuploidy, suggesting chromosomal instability which may be caused by a defective mitotic spindle checkpoint. We screened 22 glioblastomas for mutations in the mitotic spindle check-point genes hBUB1, hBUBR1 and hBUB3. DNA sequencing rev...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010100366
更新日期：2001-04-01 00:00:00
abstract：:Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00304464
更新日期：1992-01-01 00:00:00
abstract：:In this report, we describe the clinical, topographical and immunohistochemical characteristics of neurofilament (NF) inclusion formation induced by the intracisternal inoculation of young adult New Zealand white rabbits at 28-day intervals with 100 micrograms AlCl3 over the course of 267 days. The ability to recover ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294460
更新日期：1995-01-01 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：:The growth potential of 65 pituitary adenomas was determined by histochemical analysis with Ki-67 and anti-DNA polymerase alpha monoclonal antibodies, bromodeoxyuridine (BrdUdR) labeling, and counts of argyrophilic nucleolar organizer regions (Ag-NORs). The mean proliferating cell indices (PCIs) determined by Ki-67 an...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00311392
更新日期：1992-01-01 00:00:00
abstract：:This study reports the histological, ultrastructural, and immunocytochemical characteristics of intracytoplasmic eosinophilic inclusion bodies occurring in various types of neurons of the human central nervous system. By light microscopy, the inclusions were brightly eosinophilic, slightly birefringent, and sharply de...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00308960
更新日期：1989-01-01 00:00:00
abstract：:We examined the immunohistochemical localization of p35nck5a, the regulatory subunit of cyclin-dependent kinase 5 (cdk5), in brains obtained postmortem from patients with Parkinson's disease (PD) and controls. We found p35nck5a immunoreactivity in Lewy bodies (LB) in the substantia nigra, locus ceruleus, and neocortex...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050687
更新日期：1997-08-01 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：:Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a neurodegenerative disorder characterized by diabetes mellitus and optic atrophy as well as diabetes insipidus and deafness in many cases. We report the post-mortem neuropathologic findings of a patient with Wolfram syn...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0546-8
更新日期：2009-09-01 00:00:00
abstract：:Specific pathological hallmarks have been described in amyotrophic lateral sclerosis (ALS), which include motor neuronal loss, Bunina bodies (BBs) and skein like inclusions (SLIs). We investigated the relation between these three lesions in the cervical and lumbar anterior horns and the hypoglossal nuclei of 20 ALS pa...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-001-0507-3
更新日期：2002-06-01 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：:Immunohistochemical analysis of inflammatory cell density and infiltrate subpopulations in 42 meningiomas was performed. Evaluation of infiltrating cell density was carried out by cell counting. Meningothelial and fibroblastic meningiomas contained an average of 3% mononuclear cells; the few lymphocytes were localized...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00294381
更新日期：1989-01-01 00:00:00
abstract：:Electron microscopy and computerized morphometric techniques were employed to examine pericyte ultrastructure and to assess quantitatively their relationship to endothelial cells in five cases of cerebellar capillary hemangioblastoma. A total of 97 cross-sectioned capillary profiles were studied. Pericyte coverage of ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00687810
更新日期：1985-01-01 00:00:00
abstract：:Autopsy findings of a patient, with sialidosis type I phenotype carrying V217M/G243R mutations in the lysosomal sialidase gene and biochemically defined isolated sialidase deficiency, who died of intractable lymphoma at the age of 32 years, are described. Perikaryal expansion of cytoplasm was evident, mostly in motor ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-009-0544-x
更新日期：2010-01-01 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：:In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% ...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050941
更新日期：1998-12-01 00:00:00
abstract：:Neuronal and glial cytoplasmic inclusions (NCIs and GCIs), which contain alpha-synuclein as a major component, are characteristic cytopathological features of multiple system atrophy (MSA). We report MSA of 19 years' duration in a 73-year-old woman. Her initial symptom was parkinsonism, with dementia appearing about 8...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010000292
更新日期：2001-03-01 00:00:00
abstract：:Changes in morphology, behavior of the blood-brain barrier (BBB), regional cerebral blood flow (rCBF), and local cerebral glucose utilization (LCGU) were assessed and correlated in Mongolian gerbils following 5 min cerebral ischemia, produced by bilateral clamping of the common carotid arteries. The morphological chan...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00691868
更新日期：1983-01-01 00:00:00
abstract：:The neuropathological staging model of Alzheimer's disease proposed by Braak and Braak [Acta Neuropathol (1991) 82:259] requires that the evolution of neurofibrillary pathology follows a predictable pattern that can be ordered in a regular regional hierarchy. We have operationalized the neuropathological staging syste...
journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s004010050780
更新日期：1998-02-01 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
