Axonal dystrophy in the gracile nucleus in congenital biliary atresia and cystic fibrosis (mucoviscidosis): beneficial effect of vitamin E therapy.

abstract：:Peptidylarginine deiminase (PAD) and citrullinated proteins have emerged as key molecules in various human diseases, but detailed subcellular localizations of PAD2 and citrullinated proteins are poorly mapped in brain under normal and pathologic conditions. We performed subcellular fractionation and electron microscop...

journal_title：Journal of neuropathology and experimental neurology

authors： Jang B,Shin HY,Choi JK,Nguyen du PT,Jeong BH,Ishigami A,Maruyama N,Carp RI,Kim YS,Choi EK

更新日期：2011-02-01 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 content of RNA and the volume of motor neurons isolated from the lateral portion of the cervical swelling were examined in six control and six amyotrophic lateral sclerosis (ALS) cases obtained at autopsy. The mean volume of motor neurons in the ALS group did not differ significantly from the values obtained in th...

journal_title：Journal of neuropathology and experimental neurology

authors： Davidson TJ,Hartmann HA,Johnson PC

更新日期：1981-01-01 00:00:00

abstract：:The role of anti-myelin antibodies in the pathogenesis of experimental autoimmune neuritis (EAN) induced in the Lewis rat by immunization with peripheral nerve myelin has been assessed. Passive transfer with lymph node cells (LNC) or purified serum immunoglobulin from rats with EAN was employed to directly measure the...

journal_title：Journal of neuropathology and experimental neurology

authors： Taylor JM,Pollard JD

更新日期：2001-06-01 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：:Whatever the cause of myelin damage of the peripheral nervous system, the initial attack on myelin by a dysimmune process may begin either at the internodal area or in the paranodal and nodal regions. The term "nodo-paranodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to ca...

journal_title：Journal of neuropathology and experimental neurology

authors： Vallat JM,Magy L,Corcia P,Boulesteix JM,Uncini A,Mathis S

更新日期：2020-03-01 00:00:00

abstract：:Cysteine-S-sulfate is an abnormal metabolite discovered in the urine and blood of a patient with cysteine oxidase deficiency, a rare disorder of sulfur amino acid metabolism associated with brain damage and mental retardation. The molecular structure of cysteine-S-sulfate closely resembles that of glutamate and relate...

journal_title：Journal of neuropathology and experimental neurology

authors： Olney JW,Misra CH,de Gubareff T

更新日期：1975-03-01 00:00:00

abstract：:Cerebral cavernous malformations (CCMs) often cause hemorrhages that can result in severe clinical manifestations, including hemiparesis and seizures. The underlying mechanisms of the aggressive behavior of CCMs are undetermined to date, but alterations of vascular matrix components may be involved. We compared the lo...

journal_title：Journal of neuropathology and experimental neurology

authors： Schneider H,Errede M,Ulrich NH,Virgintino D,Frei K,Bertalanffy H

更新日期：2011-06-01 00:00:00

abstract：:A previous autopsy study of patients with amnestic-type mild cognitive impairment (MCI) suggested an overrepresentation of argyrophilic grain disease (AGD). We studied 34 patients who had diagnoses of amnestic MCI during progression to dementia and who came to autopsy. Neuropathologic evaluation included routine histo...

journal_title：Journal of neuropathology and experimental neurology

authors： Jicha GA,Petersen RC,Knopman DS,Boeve BF,Smith GE,Geda YE,Johnson KA,Cha R,Delucia MW,Braak H,Dickson DW,Parisi JE

更新日期：2006-06-01 00:00:00

abstract：:Microglial activation and behavioral abnormalities occur before neuronal loss in experimental murine prion disease; the behavioral changes coincide with a reduction in synaptic plasticity. Because synaptic plasticity depends on an intact perineuronal net (PN), a specialized extracellular matrix that surrounds parvalbu...

journal_title：Journal of neuropathology and experimental neurology

authors： Franklin SL,Love S,Greene JR,Betmouni S

更新日期：2008-03-01 00:00:00

abstract：:Chorea-Acanthocytosis (CHAC) is an autosomal recessive disease characterized by neurodegeneration and acanthocytosis. Enhanced creatine kinase concentration is a constant feature of the condition. The mechanism underlying CHAC is unknown. However, acanthocytosis and enhanced creatine kinase suggest a protein defect th...

journal_title：Journal of neuropathology and experimental neurology

authors： Melone MA,Di Fede G,Peluso G,Lus G,Di Iorio G,Sampaolo S,Capasso A,Gentile V,Cotrufo R

更新日期：2002-10-01 00:00:00

abstract：:Bone marrow stromal cells (BMSCs) have the potential to improve functional recovery in patients with spinal cord injury (SCI); however, they are limited by low survival rates after transplantation in the injured tissue. Our objective was to clarify the effects of a temporal blockade of interleukin 6 (IL-6)/IL-6 recept...

journal_title：Journal of neuropathology and experimental neurology

authors： Tan Y,Uchida K,Nakajima H,Guerrero AR,Watanabe S,Hirai T,Takeura N,Liu SY,Johnson WE,Baba H

更新日期：2013-10-01 00:00:00

abstract：:β-Amyloid (Aβ) plaques can trigger chronic inflammation in the cellular environment that recruits infiltrating macrophages during the course of Alzheimer disease (AD). Activated macrophages release pro-inflammatory cytokines that increase neurotoxicity associated with AD. A major impediment to investigating neuroinfla...

journal_title：Journal of neuropathology and experimental neurology

authors： Kozyrev N,Albers S,Yang J,Prado VF,Prado MAM,Fonseca GJ,Rylett RJ,Dekaban GA

更新日期：2020-11-01 00:00:00

abstract：:Mucopolysaccharidosis IIID (MPS IIID) is one of the rarest of the MPS-III syndromes. To date, the clinical manifestations of 10 patients have been reported, the deficient N-acetylglucosamine 6-sulfatase (G6S) enzyme has been purified, and the G6S gene has been cloned, sequenced and localized. However, morphological ma...

journal_title：Journal of neuropathology and experimental neurology

authors： Jones MZ,Alroy J,Rutledge JC,Taylor JW,Alvord EC Jr,Toone J,Applegarth D,Hopwood JJ,Skutelsky E,Ianelli C,Thorley-Lawson D,Mitchell-Herpolsheimer C,Arias A,Sharp P,Evans W,Sillence D,Cavanagh KT

更新日期：1997-10-01 00:00:00

abstract：:The occurrence of neuroinflammation after spinal cord injury (SCI) is well established, but its function is debated, with both beneficial and detrimental consequences ascribed. A discriminate of the role of neuroinflammation may be the time period after SCI, and there is evidence to favor early neuroinflammation being...

journal_title：Journal of neuropathology and experimental neurology

authors： Rice T,Larsen J,Rivest S,Yong VW

更新日期：2007-03-01 00:00:00

abstract：:The roles of bone morphogenetic proteins (BMPs) and their receptors (BMPRs) in meningioma biology are not known. In this study, frozen tissues from 26 World Health Organization Grades I to III meningiomas were analyzed by Western blot for BMP-2/4, BMPR IA, and BMPR II, and activation of downstream p-Smad1, p38 mitogen...

journal_title：Journal of neuropathology and experimental neurology

authors： Johnson MD,O'Connell MJ,Vito F,Pilcher W

更新日期：2009-11-01 00:00:00

abstract：:Lysosomal beta-hexosaminidase consists of 2 subunits, alpha and beta. Mutations in the alpha-subunit gene cause Tay-Sachs disease, while mutations in the beta-subunit gene cause Sandhoff disease. Mice generated by targeted disruption of either the alpha- or beta-subunit genes displayed the pathological features of Tay...

journal_title：Journal of neuropathology and experimental neurology

authors： Suzuki K,Sango K,Proia RL,Langaman C

更新日期：1997-06-01 00:00:00

abstract：:The formation of Lewy bodies (LBs) and their relationship to other types of nigral inclusions associated with Parkinson disease (PD), such as pale bodies (PBs), remain poorly understood. Known constituents of LBs include alpha-synuclein (alphaS) and ubiquitin (Ub), providing windows to their morphogenesis. Additionall...

journal_title：Journal of neuropathology and experimental neurology

authors： Kuusisto E,Parkkinen L,Alafuzoff I

更新日期：2003-12-01 00:00:00

abstract：:We report a novel case of a World Health Organization grade 3 anaplastic meningioma arising from the olfactory groove in an 83-year-old woman. Molecular and methylation profiling confirm this lesion to be an NF2 subtype, methylation class intermediate type B meningioma. As most meningiomas in this location are indolen...

journal_title：Journal of neuropathology and experimental neurology

authors： Foo ASC,Tan DTM,Tan CL,Sahm F,von Deimling A,Yeo TT

更新日期：2017-12-01 00:00:00

abstract：:Lhermitte-Duclos disease (LDD) is a rare cerebellar tumor associated with Cowden disease (CD) and germline mutations in the PTEN gene. To further define these relationships, we reviewed clinical and pathologic findings in 31 LDD cases and analyzed the status of the PTEN pathway in 11 of them. We hypothesized that the ...

journal_title：Journal of neuropathology and experimental neurology

authors： Abel TW,Baker SJ,Fraser MM,Tihan T,Nelson JS,Yachnis AT,Bouffard JP,Mena H,Burger PC,Eberhart CG

更新日期：2005-04-01 00:00:00

abstract：:A working group supported by the Office of Rare Diseases of the National Institutes of Health formulated neuropathologic criteria for corticobasal degeneration (CBD) that were subsequently validated by an independent group of neuropathologists. The criteria do not require a specific clinical phenotype, since CBD can h...

journal_title：Journal of neuropathology and experimental neurology

authors： Dickson DW,Bergeron C,Chin SS,Duyckaerts C,Horoupian D,Ikeda K,Jellinger K,Lantos PL,Lippa CF,Mirra SS,Tabaton M,Vonsattel JP,Wakabayashi K,Litvan I,Office of Rare Diseases of the National Institutes of Health.

更新日期：2002-11-01 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

abstract：:Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder associated with repetitive traumatic brain injury. Multiple system atrophy (MSA) is a Parkinsonian disorder that can result in repetitive falls with associated head trauma. We hypothesized that patients with neurodegenerative disorders ...

journal_title：Journal of neuropathology and experimental neurology

authors： Koga S,Dickson DW,Bieniek KF

更新日期：2016-10-01 00:00:00

abstract：:Glioblastoma (GBM), representing WHO grade IV astrocytoma, is a relatively common primary brain tumor in adults with an exceptionally dismal prognosis. With an incidence rate of over 10 000 cases in the United States annually, the median survival rate ranges from 10-15 months in IDH1/2-wildtype tumors and 24-31 months...

journal_title：Journal of neuropathology and experimental neurology

authors： Richardson TE,Patel S,Serrano J,Sathe AA,Daoud EV,Oliver D,Maher EA,Madrigales A,Mickey BE,Taxter T,Jour G,White CL,Raisanen JM,Xing C,Snuderl M,Hatanpaa KJ

更新日期：2019-06-01 00:00:00

abstract：:Brain arteriovenous malformations (bAVMs) are congenital anomalies of blood vessels that cause intracranial hemorrhage in children and young adults. Chromosomal rearrangements and fusion genes play an important role in tumor pathogenesis, though the role of fusion genes in bAVM pathophysiological processes is unclear....

journal_title：Journal of neuropathology and experimental neurology

authors： Yan Z,Fan G,Li H,Jiao Y,Fu W,Weng J,Huo R,Wang J,Xu H,Wang S,Cao Y,Zhao J

更新日期：2021-01-01 00:00:00

abstract：:Experimental autoimmune neuritis (EAN) is a demyelinating disease of the peripheral nervous system (PNS). This acute inflammatory disease is mediated by CD4+ T cells and bears significant similarities to the Guillain-Barré syndrome of humans. In the present study, we investigated the function of IL-18 in T cell-mediat...

journal_title：Journal of neuropathology and experimental neurology

authors： Yu S,Chen Z,Mix E,Zhu SW,Winblad B,Ljunggren HG,Zhu J

更新日期：2002-07-01 00:00:00

abstract：:We previously found that vascular smooth muscle actin (SMA) is reduced in the brains of patients with late stage Alzheimer disease (AD) compared with brains of nondemented, neuropathologically normal subjects. To assess the pathogenetic significance and disease specificity of this finding, we studied 3 additional pati...

journal_title：Journal of neuropathology and experimental neurology

authors： Hulette CM,Ervin JF,Edmonds Y,Antoine S,Stewart N,Szymanski MH,Hayden KM,Pieper CF,Burke JR,Welsh-Bohmer KA

更新日期：2009-04-01 00:00:00

abstract：:Vanishing white matter disease (VWM) is a progressive cavitating disease of central white matter due to a deficiency of the translation initiation factor eIF2B. Oligodendrocytes appear to be numerically increased in some white matter areas, while decreased in others. We compared oligodendrocytes of cerebral, cerebella...

journal_title：Journal of neuropathology and experimental neurology

authors： Van Haren K,van der Voorn JP,Peterson DR,van der Knaap MS,Powers JM

更新日期：2004-06-01 00:00:00

abstract：:Mutations in the cathepsin D (CTSD) gene cause an aggressive neurodegenerative disease (congenital neuronal ceroid lipofuscinosis) that leads to early death. Recent evidence suggests that presynaptic abnormalities play a major role in the pathogenesis of CTSD deficiencies. To identify the early events that lead to syn...

journal_title：Journal of neuropathology and experimental neurology

authors： Koch S,Molchanova SM,Wright AK,Edwards A,Cooper JD,Taira T,Gillingwater TH,Tyynelä J

更新日期：2011-12-01 00:00:00

abstract：:To determine whether arterial wall degeneration, in combination with hemodynamic insult, causes cerebral artery aneurysms in a dog model, we simulated the geometry and hemodynamics of a human artery by surgical reconstruction of both common carotid arteries in 12 dogs. The dogs were then randomly assigned to one of th...

journal_title：Journal of neuropathology and experimental neurology

authors： Zhu YQ,Li MH,Yan L,Tan HQ,Cheng YS

更新日期：2014-09-01 00:00:00