Expression of cell adhesion molecules in inflammatory myopathies and Duchenne dystrophy.

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：:Nemaline myopathy is the most common congenital myopathy and is caused by mutations in various genes such as ACTA1 (encoding skeletal α-actin). It is associated with limb and respiratory muscle weakness. Despite increasing clinical and scientific interest, the molecular and cellular events leading to such weakness rem...

journal_title：Journal of neuropathology and experimental neurology

authors： Lindqvist J,Cheng AJ,Renaud G,Hardeman EC,Ochala J

更新日期：2013-06-01 00:00:00

abstract：:Interferon-stimulated gene 15 (ISG15), an antagonist of the ubiquitin pathway, is elevated in cells and brain tissues obtained from ataxia telangiectasia (A-T) patients. Previous studies reveal that an elevated ISG15 pathway inhibits ubiquitin-dependent protein degradation, leading to activation of basal autophagy as ...

journal_title：Journal of neuropathology and experimental neurology

authors： Kim CD,Reed RE,Juncker MA,Fang Z,Desai SD

更新日期：2017-07-01 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：:Due to recent biological and technical advances, the list of potentially useful candidate genes is rapidly expanding in the study of brain tumors. However, traditional methods of screening individual genes in individual samples are slow and tedious, often with consumption of precious resources after only a few experim...

journal_title：Journal of neuropathology and experimental neurology

authors： Fuller CE,Wang H,Zhang W,Fuller GN,Perry A

更新日期：2002-12-01 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：: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：: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：:How to assess the substantial neuronal loss in a neurodegenerative disease such as Alzheimer disease is still being debated. Recently, stereological procedures have been proposed that claim improved accuracy and statistical power, but the results of some of these investigations have been controversial. In this study w...

journal_title：Journal of neuropathology and experimental neurology

authors： Everall IP,DeTeresa R,Terry R,Masliah E

更新日期：1997-11-01 00:00:00

abstract：:The oligoastrocytoma, as a mixed glioma, represents a nosologic dilemma with respect to precisely defining the oligodendroglial and astroglial phenotypes that constitute the neoplastic cell lineages of these tumors. In this study, cell image analysis with Feulgen-stained nuclei was used to distinguish between oligoden...

journal_title：Journal of neuropathology and experimental neurology

authors： Decaestecker C,Lopes BS,Gordower L,Camby I,Cras P,Martin JJ,Kiss R,VandenBerg SR,Salmon I

更新日期：1997-04-01 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：: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：:Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performe...

journal_title：Journal of neuropathology and experimental neurology

authors： Hirano A,Nakano I,Kurland LT,Mulder DW,Holley PW,Saccomanno G

更新日期：1984-09-01 00:00:00

abstract：:Inclusion body myositis (IBM) is a late-onset inflammatory myopathy with distinctive clinical and histopathological features. The molecular basis for the disease remains unknown, but abnormal nuclear morphology and the accumulation of a protein that binds single-stranded DNA in a sequence-independent fashion suggest a...

journal_title：Journal of neuropathology and experimental neurology

authors： Horvath R,Fu K,Johns T,Genge A,Karpati G,Shoubridge EA

更新日期：1998-05-01 00:00:00

abstract：:The relevance of cerebral amyloid angiopathy (CAA) to the pathogenesis of Alzheimer disease (AD) and dementia in general emphasizes the importance of developing novel targeting approaches for detecting and treating cerebrovascular amyloid (CVA) deposits. We developed a nanoparticle-based technology that uses a monoclo...

journal_title：Journal of neuropathology and experimental neurology

authors： Poduslo JF,Hultman KL,Curran GL,Preboske GM,Chamberlain R,Marjańska M,Garwood M,Jack CR Jr,Wengenack TM

更新日期：2011-08-01 00:00:00

abstract：:Much of the morbidity after traumatic brain injury (TBI) is associated with traumatic axonal injury (TAI). Although most TAI studies focus on corpus callosum white matter, the visual system has received increased interest. To assess visual system TAI, we developed a mouse model of optic nerve TAI. It is unknown, howev...

journal_title：Journal of neuropathology and experimental neurology

authors： Wang J,Fox MA,Povlishock JT

更新日期：2013-08-01 00:00:00

abstract：:Frontotemporal dementia with parkinsonism (FTDP-17) is an autosomal dominant disorder that presents clinically with dementia, extrapyramidal signs, and behavioral disturbances in mid-life and progresses to death within 5 to 10 years. Pathologically, the disorder is characterized by variable neuronal loss and gliosis i...

journal_title：Journal of neuropathology and experimental neurology

authors： Hulette CM,Pericak-Vance MA,Roses AD,Schmechel DE,Yamaoka LH,Gaskell PC,Welsh-Bohmer KA,Crowther RA,Spillantini MG

更新日期：1999-08-01 00:00:00

abstract：:Molecular pathology has identified 2 distinct forms of neuronal inclusion body in Amyotrophic Lateral Sclerosis (ALS). ALS-type inclusions are skeins or small dense filamentous aggregates which can only be demonstrated by ubiquitin immunocytochemistry (ICC). In contrast hyaline conglomerates (HC) are large multifocal ...

journal_title：Journal of neuropathology and experimental neurology

authors： Ince PG,Tomkins J,Slade JY,Thatcher NM,Shaw PJ

更新日期：1998-10-01 00:00:00

abstract：:The effects of hypoglycemia on cerebrovascular permeability to a protein, horseradish peroxidase (HRP), were studied in mice given 3 or 8 units of crytalline zinc insulin intraperitoneally. HRP (10 mg in 0.1 ml saline) was injected intravenously 15 to 20 minutes prior to sacrifice. Both mildly and severely hypoglycemi...

journal_title：Journal of neuropathology and experimental neurology

authors： Hsu DW,Hedley-Whyte ET

更新日期：1980-05-01 00:00:00

abstract：:To evaluate cognitive performance among persons who did and did not develop clinical Alzheimer disease (AD) but had AD neuropathology at autopsy, we examined neuropsychological performance in cognitively healthy (Clinical Dementia Rating [CDR] = 0) participants who returned for at least 1 follow-up and died within 2 y...

journal_title：Journal of neuropathology and experimental neurology

authors： Hassenstab J,Monsell SE,Mock C,Roe CM,Cairns NJ,Morris JC,Kukull W

更新日期：2015-11-01 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：:X-linked Charcot-Marie-Tooth disease (CMTX) is an inherited demyelinating neuropathy caused by mutations in the gene encoding the gap junction protein connexin32 (Cx32). Despite the identification of over 160 different mutations in the Cx32 coding sequence, it is not known whether the mutations cause the disease manif...

journal_title：Journal of neuropathology and experimental neurology

authors： Abel A,Bone LJ,Messing A,Scherer SS,Fischbeck KH

更新日期：1999-07-01 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：:We investigated the temporal course of blood-nerve barrier (BNB) breakdown during the evolution of tellurium neuropathy, ricin neuropathy, and Wallerian degeneration following nerve transection or nerve crush. Blood-nerve barrier permeability was assessed with a 4,000-molecular weight fluoresceinated dextran from thre...

journal_title：Journal of neuropathology and experimental neurology

authors： Bouldin TW,Earnhardt TS,Goines ND

更新日期：1991-11-01 00:00:00

abstract：:A primary cerebellar rhabdomyosarcoma (RMS) in a six and a half year old boy is reported. Microscopy of the surgical material revealed lobules of closely packed cells with a high mitotic rate, pleomorphic hyperchromatic nuclei and scant cytoplasm. At their periphery, the lobules merged with rounded cells with similar ...

journal_title：Journal of neuropathology and experimental neurology

authors： Hinton DR,Halliday WC

更新日期：1984-07-01 00:00:00

abstract：:Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in the amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor the extent of P...

journal_title：Journal of neuropathology and experimental neurology

authors： Riva N,Chaabane L,Peviani M,Ungaro D,Domi T,Dina G,Bianchi F,Spano G,Cerri F,Podini P,Corbo M,Carro UD,Comi G,Bendotti C,Quattrini A

更新日期：2014-07-01 00:00:00

abstract：:Although stroke is among the most common causes of death and chronic disability worldwide, the proteome of the ischemic human brain remains unknown. Only a few studies have investigated the ischemic brain proteome in rodent stroke models. We performed a proteomic study of the human brain after ischemic stroke using a ...

journal_title：Journal of neuropathology and experimental neurology

authors： Cuadrado E,Rosell A,Colomé N,Hernández-Guillamon M,García-Berrocoso T,Ribo M,Alcazar A,Ortega-Aznar A,Salinas M,Canals F,Montaner J

更新日期：2010-11-01 00:00:00

abstract：:Acetylcholinesterase (AChE) activity has been studied in normal, control and denervated muscle of rabbits by electron microscopic-cytochemistry and radiometric assay. A small amount of butyrylcholinesterase (BuChE) activity is also found in biochemical assay of unfixed muscle, but it is not demonstrable cytochemically...

journal_title：Journal of neuropathology and experimental neurology

authors： Tennyson VM,Kremzner LT,Brzin M

更新日期：1977-03-01 00:00:00

abstract：:Hereditary gelsolin amyloidosis (AGel amyloidosis) is a systemic disorder reported worldwide in kindreds with a G654A or G654T gelsolin gene mutation. The clinically characteristic peripheral nerve involvement has been poorly characterized morphologically, and its pathogenesis remains unknown. We studied peripheral ne...

journal_title：Journal of neuropathology and experimental neurology

authors： Kiuru-Enari S,Somer H,Seppäläinen AM,Notkola IL,Haltia M

更新日期：2002-06-01 00:00:00

abstract：:Nicotinic acetylcholine receptors (nAChRs) mediate fast synaptic transmission in autonomic ganglia, which innervate and control the activity of most visceral organs. By combining ultrastructural, immunocytochemical, and pharmacological analyses, we characterized the nAChR subtypes in the rat superior cervical ganglion...

journal_title：Journal of neuropathology and experimental neurology

authors： Del Signore A,Gotti C,Rizzo A,Moretti M,Paggi P

更新日期：2004-02-01 00:00:00