Abnormal accumulation of tTGase products in muscle and erythrocytes of chorea-acanthocytosis patients.

abstract：:Placement of a freezing probe on the skull of neonatal rats produces four-layered microgyria, complete with a lamina dissecans and microsulcus. We studied the developmental course of this induced microgyria under light microscopy by examining changes in neurons, glia, and macrophages following a focal freezing insult ...

journal_title：Journal of neuropathology and experimental neurology

authors： Rosen GD,Press DM,Sherman GF,Galaburda AM

更新日期：1992-11-01 00:00:00

abstract：:Neuron-derived molecules are potent regulators of oligodendrocyte differentiation and myelination during brain development and upon demyelination. Their analysis will thus contribute to understanding remyelination failure in demyelinating diseases, such as multiple sclerosis (MS). Previously, we have identified neuron...

journal_title：Journal of neuropathology and experimental neurology

authors： de Jong CGHM,Stancic M,Pinxterhuis TH,van Horssen J,van Dam AM,Gabius HJ,Baron W

更新日期：2018-11-01 00:00:00

abstract：:The presence of intermediate filaments (IF) (diameter 10 nm) is a characteristic electron microscopic finding in the cytoplasm of meningioma cells. To identify these IF, immunohistochemical staining for cytokeratins and vimentin and two-dimensional (2-D) gel electrophoresis followed by immunoblot analysis were applied...

journal_title：Journal of neuropathology and experimental neurology

authors： Halliday WC,Yeger H,Duwe GF,Phillips MJ

更新日期：1985-11-01 00:00:00

abstract：:A superoxide dismutase 1 (SOD-1)genetic defect has been identified in familial amyotrophic lateral sclerosis (ALS) and motor neuron degeneration has been described in SOD-1 transgenic mice. Because an excitotoxic mechanism has been implicated in ALS, we undertook studies to provide a description of excitotoxic degener...

journal_title：Journal of neuropathology and experimental neurology

authors： Ikonomidou C,Qin Qin Y,Labruyere J,Olney JW

更新日期：1996-02-01 00:00:00

abstract：:Pediatric and NF2-associated meningiomas are uncommon and poorly characterized in comparison to sporadic adult cases. In order to elucidate their molecular features, we analyzed MIB-1, progesterone receptor (PR), NF2, merlin, DAL-1, DAL-1 protein, and chromosomal arms 1p and 14q in 53 meningiomas from 40 pediatric/NF2...

journal_title：Journal of neuropathology and experimental neurology

authors： Perry A,Giannini C,Raghavan R,Scheithauer BW,Banerjee R,Margraf L,Bowers DC,Lytle RA,Newsham IF,Gutmann DH

更新日期：2001-10-01 00:00:00

abstract：:Neurofilamentous axonal swellings occur in a number of degenerative and toxic disorders of the nervous system. In one of these, experimental intoxication with beta, beta'-iminodiproprionitrile (IDPN), accumulation of neurofilaments has been shown to result from a defect in slow axonal transport. The consequence of thi...

journal_title：Journal of neuropathology and experimental neurology

authors： Clark AW,Griffin JW,Price DL

更新日期：1980-01-01 00:00:00

abstract：:Two basic proteins, P1 of molecular weight 14,200 and P2 of molecular weight 12,300, purified from bovine peripheral nerve, were assayed for biological activity. The P1 protein is an exclusively neuritogenic agent, capable of producing clinical signs of experimental allergic neuritis (EAN) and histological abnormaliti...

journal_title：Journal of neuropathology and experimental neurology

authors： Abramsky O,Teitelbaum D,Webb C,Arnon R

更新日期：1975-01-01 00:00:00

abstract：:Primary central nervous system lymphomas (PCNSLs) are more resistant to radiotherapy and chemotherapy in AIDS (A-PCNSLs) than in non-AIDS patients (NA-PCNSLs). We investigated 23 A-PCNSLs and 24 NA-PCNSLs. Lymphoma cell kinetics (i.e. proliferation [mitotic index, MIB-1 and PCNA labeling indices], apoptosis and turnov...

journal_title：Journal of neuropathology and experimental neurology

authors： Christov C,Adle-Biassette H,Lechapt E,Poron F,Gray F,Gaulard P,Gherardi RK

更新日期：1999-10-01 00:00:00

abstract：:The blood-brain barrier (BBB) is recognized as a barrier to the trafficking of molecules and cellular elements into the central nervous system (CNS). Horseradish peroxidase (HRP) exclusion is used as a measure of BBB integrity. The BBB is altered and becomes permeable during the course of experimental allergic encepha...

journal_title：Journal of neuropathology and experimental neurology

authors： Knobler RL,Marini JC,Goldowitz D,Lublin FD

更新日期：1992-01-01 00:00:00

abstract：:Canine smooth muscle cells (SMCs), cultured from amyloid-affected brain blood vessels accumulate Alzheimer amyloid-beta peptide (Abeta) intracellularly, either spontaneously or after treatment with apolipoprotein E (apoE). ApoE is codeposited with Abeta, which suggests that apoE participates in Abeta accumulation. We ...

journal_title：Journal of neuropathology and experimental neurology

authors： Mazur-Kolecka B,Frackowiak J,Krzeslowska J,Ramakrishna N,Haske T,Emmerling MR,Zhang W,Kim KS,Wisniewski HM

更新日期：1999-03-01 00:00:00

abstract：:PROX1 is a prospero-related transcription factor that plays a critical role in the development of various organs including the mammalian lymphatic and central nervous systems; it controls cell proliferation and differentiation through different transcription pathwaysand has both oncogenic and tumor-suppressive functio...

journal_title：Journal of neuropathology and experimental neurology

authors： Elsir T,Eriksson A,Orrego A,Lindström MS,Nistér M

更新日期：2010-02-01 00:00:00

abstract：:We describe the clinical, pathological, ultrastructural and biochemical features in the case of a 15-year-old boy with multiple sulfatase deficiency. Clinical abnormalities included hypotonia, retarded psychomotor development, hepatosplenomegaly, pigmentary degeneration of the retina, myoclonic seizures, aortic insuff...

journal_title：Journal of neuropathology and experimental neurology

authors： Guerra WF,Verity MA,Fluharty AL,Nguyen HT,Philippart M

更新日期：1990-07-01 00:00:00

abstract：:Brain atrophy is a common finding in patients with AIDS, but the relationship of atrophy to HIV-associated dementia is unclear. We used unbiased, stereological methods on postmortem brain specimens to estimate volumes of different brain regions in patients prospectively diagnosed with and without HIV-associated dement...

journal_title：Journal of neuropathology and experimental neurology

authors： Subbiah P,Mouton P,Fedor H,McArthur JC,Glass JD

更新日期：1996-10-01 00:00:00

abstract：:Retinoblastoma protein (pRb) is a ubiquitous 928-amino acid cell cycle regulatory molecule with diverse biologic activities. One critical function of pRb is the control of the G1-to-S phase checkpoint of the cell cycle. In the hypophosphorylated state, pRb suppresses the activity of E2F transcription factors thereby i...

journal_title：Journal of neuropathology and experimental neurology

authors： Stone JG,Siedlak SL,Tabaton M,Hirano A,Castellani RJ,Santocanale C,Perry G,Smith MA,Zhu X,Lee HG

更新日期：2011-07-01 00:00:00

abstract：:Retraction of distal sensory axons is a prominent feature in diabetic peripheral neuropathy (DPN), a process amenable to insulin therapy. Nevertheless, diabetic patients and long-term diabetic mice develop motor deficits after longer durations of DPN, a process that may be related to insulin deficiency. To compare the...

journal_title：Journal of neuropathology and experimental neurology

authors： Francis GJ,Martinez JA,Liu WQ,Zochodne DW,Hanson LR,Frey WH 2nd,Toth C

更新日期：2011-05-01 00:00:00

abstract：:It is recognized that IDH mutation negative, low-grade epilepsy associated tumors (LEAT) can show diffuse growth patterns and lack the diagnostic hallmarks of either classical dysembryoplastic neuroepithelial tumors (DNT) or typical ganglioglioma. "Nonspecific or diffuse DNT" and more recently "polymorphous low-grade ...

journal_title：Journal of neuropathology and experimental neurology

authors： Al-Hajri A,Al-Mughairi S,Somani A,An S,Liu J,Miserocchi A,McEvoy AW,Yousry T,Hoskote C,Thom M

更新日期：2017-12-01 00:00:00

abstract：:This is a report of an unusual, densely cellular, midcerebellar neoplasm in a seven-year-old boy. Although clinically consistent with a medulloblastoma, immunohistochemistry and electron microscopy demonstrated glial and rhabdomyoblastic differentiation in the tumor. We discuss the differential diagnosis of this tumor...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Hart MN,Menezes A,Cancilla PA

更新日期：1983-11-01 00:00:00

abstract：:Intracranial hemangiopericytic meningiomas (HM) from seven patients were examined by immunostaining, transmission electron microscopy (TEM), scanning electron microscopy (SEM) and cell culture. Positive staining for Factor VIII-related antigen was restricted to capillary endothelial cells. There was no reaction with a...

journal_title：Journal of neuropathology and experimental neurology

authors： Nakamura M,Inoue HK,Ono N,Kunimine H,Tamada J

更新日期：1987-01-01 00:00:00

abstract：:In Niemann-Pick Type C (NPC) disease, the concentration of cholesterol increases with age in every tissue except the brain. This study investigates whether accumulation of cholesterol might also occur within the cells of the central nervous system (CNS), but be obscured by the simultaneous loss of sterol from myelin a...

journal_title：Journal of neuropathology and experimental neurology

authors： Xie C,Burns DK,Turley SD,Dietschy JM

更新日期：2000-12-01 00:00:00

abstract：:The most common histologic feature in patients with frontotemporal lobar degeneration (FTLD) is intracellular brain inclusions of yet uncharacterized proteins that react with antiubiquitin (Ub) antibodies, but not with tau or synuclein (FTLD-U). We identified a four-generation Belgian FTLD family in which 8 patients h...

journal_title：Journal of neuropathology and experimental neurology

authors： Pirici D,Vandenberghe R,Rademakers R,Dermaut B,Cruts M,Vennekens K,Cuijt I,Lübke U,Ceuterick C,Martin JJ,Van Broeckhoven C,Kumar-Singh S

更新日期：2006-03-01 00:00:00

abstract：:The diagnosis of Alzheimer disease (AD) according to current criteria is a combined clinical and pathological exercise. The clinical discrimination of AD from other types of dementia may be complicated when the patient suffers from more than one disease. In particular the concomitant presence of other neurological con...

journal_title：Journal of neuropathology and experimental neurology

authors： Nagy Z,Esiri MM,Jobst KA,Morris JH,King EM,McDonald B,Joachim C,Litchfield S,Barnetson L,Smith AD

更新日期：1997-02-01 00:00:00

abstract：:Glomeruloid bodies (GBs), tumor-associated vascular structures with a superficial resemblance to renal glomeruli, are important histopathological features of glioblastoma multiforme, but have also been described in other types of tumors and in cerebral metastases. The purpose of this study was to elucidate the pathoge...

journal_title：Journal of neuropathology and experimental neurology

authors： Döme B,Tímár J,Paku S

更新日期：2003-06-01 00:00:00

abstract：:Human neurodegenerative diseases can be characterized as disorders of protein aggregation. As a key player in cellular autophagy and the ubiquitin proteasome system, p62 may represent an effective immunohistochemical target, as well as mechanistic operator, across neurodegenerative proteinopathies. In this study, 2 no...

journal_title：Journal of neuropathology and experimental neurology

authors： Trejo-Lopez JA,Sorrentino ZA,Riffe CJ,Prokop S,Dickson DW,Yachnis AT,Giasson BI

更新日期：2020-04-01 00:00:00

abstract：:Brain parenchymal involvement of mycosis fungoides (MF) is very rare. This study reports a patient with known cutaneous MF (under treatment) who presented with a CNS syndrome and multiple brain lesions. Brain biopsy demonstrated massive eosinophilic infiltrates but no MF cells. Despite treatment, new lesions developed...

journal_title：Journal of neuropathology and experimental neurology

authors： Schowinsky J,Leppert M,Ney D,Kleinschmidt-DeMasters BK

更新日期：2015-10-01 00:00:00

abstract：:Rupture of a saccular intracranial aneurysm (sIA) is often fatal. Thus, early detection of rupture-prone sIAs is vital. Myeloperoxidase (MPO), derived mainly from neutrophils, associates with sIA rupture, and therefore its role in sIA pathogenesis warrants further studies. We analyzed MPO and its association with othe...

journal_title：Journal of neuropathology and experimental neurology

authors： Ollikainen E,Tulamo R,Lehti S,Hernesniemi J,Niemelä M,Kovanen PT,Frösen J

更新日期：2018-06-01 00:00:00

abstract：:Mutations in the parkin gene are the most common cause of early-onset autosomal recessive Parkinson disease (PD). The pathogenic mechanisms of how parkin mutations lead to the development of PD are not fully understood. Studies of cell cultures and of Drosophila have suggested a dominant negative effect for the clinic...

journal_title：Journal of neuropathology and experimental neurology

authors： Van Rompuy AS,Lobbestael E,Van der Perren A,Van den Haute C,Baekelandt V

更新日期：2014-02-01 00:00:00

abstract：:The idiopathic inflammatory myopathies (IIMs) dermatomyositis, polymyositis, and inclusion body myositis are characterized by myofiber degeneration and inflammation. The triggering factors of muscle autoaggression in these disorders are unknown, but infiltrating T cells may be activated locally and proliferate in situ...

journal_title：Journal of neuropathology and experimental neurology

authors： Bernasconi P,Cappelletti C,Navone F,Nessi V,Baggi F,Vernos I,Romaggi S,Confalonieri P,Mora M,Morandi L,Mantegazza R

更新日期：2008-06-01 00:00:00

abstract：:A noninvasive photodynamic method has been developed to produce focal brain necrosis using porphyrin activated in vivo with laser light. After peripheral injection of the photosensitive porphyrin derivative, Photofrin I, mice were irradiated on the posterior lateral aspect of the head through the intact depilated scal...

journal_title：Journal of neuropathology and experimental neurology

authors： Stroop WG,Battles EJ,Townsend JJ,Schaefer DC,Baringer JR,Straight RC

更新日期：1989-09-01 00:00:00

abstract：:Since first described, amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of Guam has represented an important model of age-related neurodegenerative disease. ALS/PDC is characterized neuropathologically by severe widespread involvement by neurofibrillary tangles (NFTs). Over the past 30 years there...

journal_title：Journal of neuropathology and experimental neurology

authors： Perl DP,Hof PR,Purohit DP,Loerzel AJ,Kakulas BA

更新日期：2003-04-01 00:00:00

abstract：:Increasing evidence has demonstrated a vital role of microRNAs (miRNAs) in diverse biological processes. However, their functions in developing brain with hypoxia-ischemia (HI) remain largely unknown. Through a miRNA microarray analysis in a P10 rat model of cerebral HI, we found that miR-30d-5p was one of the most de...

journal_title：Journal of neuropathology and experimental neurology

authors： Zhao F,Qu Y,Zhu J,Zhang L,Huang L,Liu H,Li S,Mu D

更新日期：2017-08-01 00:00:00