Abnormalities in early markers of muscle involvement support a delay in myogenesis in spinal muscular atrophy.

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

abstract：:In 63 patients with malabsorption syndromes, 16 with congenital biliary atresia (BA) and 47 with cystic fibrosis (CF), axonal dystrophy in the gracile nucleus (ADG) was studied. Of the 16 patients with BA, ADG of considerable severity was observed in all 10 over one year of age. Of the 47 patients with CF, it was obse...

journal_title：Journal of neuropathology and experimental neurology

authors： Sung JH,Park SH,Mastri AR,Warwick WJ

更新日期：1980-09-01 00:00:00

abstract：:Cutaneous and leptomeningeal vascular malformations are hallmarks of the Sturge-Weber Syndrome (SWS), resulting in chronic ischemic tissue damage. The mechanisms underlying the pathobiology of these progressive lesions are unknown. Aberrant expression of angiogenic factors has been implicated in the genesis and mainte...

journal_title：Journal of neuropathology and experimental neurology

authors： Comati A,Beck H,Halliday W,Snipes GJ,Plate KH,Acker T

更新日期：2007-01-01 00:00:00

abstract：:The development and differentiation of oligodendrocytes (OC) in developing mouse spinal cord (MSC) were investigated by correlative analysis of light and electron microscopy (EM), and immunoperoxidase studies for glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP). The sequential development of glial...

journal_title：Journal of neuropathology and experimental neurology

authors： Choi BH

更新日期：1986-09-01 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：:Most malignant human tumors display a high degree of intratumoral heterogeneity at the time of diagnosis that contributes to treatment failure. This also applies to malignant peripheral nerve sheath tumors (MPNSTs) and aggressive soft tissue sarcomas that arise sporadically or in the context of neurofibromatosis type ...

journal_title：Journal of neuropathology and experimental neurology

authors： Koelsch B,van den Berg L,Grabellus F,Fischer C,Kutritz A,Kindler-Röhrborn A

更新日期：2013-05-01 00:00:00

abstract：:In order to induce a non-lethal infection restricted to central aminergic neurons projecting to the olfactory bulbs a series of temperature sensitive (ts) and G-protein monoclonal antibody escape mutants of vesicular stomatitis virus (VSV) were instilled into the nasal cavity of mice. In three-week (wk)-old NMRI mice ...

journal_title：Journal of neuropathology and experimental neurology

authors： Lundh B,Löve A,Kristensson K,Norrby E

更新日期：1988-09-01 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

abstract：:The immunopathological findings and their effects upon the vascular permeability of the ciliary process, choroid plexus and renal glomeruli to intravenously injected 125I-bovine serum albumin (BSA) have been studied in 26 rats who survived a prolonged period of bovine serum albuminemia following the experimental chron...

journal_title：Journal of neuropathology and experimental neurology

authors： Peress NS,Miller F,Palu W

更新日期：1977-07-01 00:00:00

abstract：:Numerous studies have established the key role of the Golgi apparatus (GA) in post-translational processing, transport and targeting of proteins destined for secretion, lysosomes and plasma membranes. Moreover, several studies performed in our laboratories have shown that the size of the immunocytochemically detected ...

journal_title：Journal of neuropathology and experimental neurology

authors： Salehi A,Ravid R,Gonatas NK,Swaab DF

更新日期：1995-09-01 00:00:00

abstract：:The role of axonal transport in the pathogenesis of the axonal swellings which develop at the severed ends of transected axons was studied by electron microscopic (EM) autoradiography. Proteins carried by fast anterograde transport in rat sciatic nerves were labeled with [3H]-leucine or [3H]-fucose; [3H]-leucine, [3H]...

journal_title：Journal of neuropathology and experimental neurology

authors： Griffin JW,Price DL,Engel WK,Drachman DB

更新日期：1977-03-01 00:00:00

abstract：:The development of deformities during the course of leprosy disease is a major public health concern worldwide. It is possible that cytokine production and apoptosis of Schwann cells (SCs) directly affect nerve degeneration and regeneration leading to injury of the myelin sheath and axon. In the present study, the exp...

journal_title：Journal of neuropathology and experimental neurology

authors： Oliveira RB,Sampaio EP,Aarestrup F,Teles RM,Silva TP,Oliveira AL,Antas PR,Sarno EN

更新日期：2005-10-01 00:00:00

abstract：:Long-term administration of chloroquine to rats induces a vacuolar myopathy, which is specifically termed chloroquine myopathy (CM). In CM, tau mRNA levels were transiently upregulated in the early phase, while tau itself slowly accumulated in the late phase. The temporal profiles of tau mRNA levels and its accumulati...

journal_title：Journal of neuropathology and experimental neurology

authors： Murakami N,Oyama F,Gu Y,McLennan IS,Nonaka I,Ihara Y

更新日期：1998-07-01 00:00:00

abstract：:In adult cerebral astrocytomas, the more anaplasia that is present, the more malignancy that occurs. Cell proliferation antigen staining (MIB-1) and DNA labeling methods for apoptosis using paraffin sections from 39 cases were compared with histopathological grading systems for predicting patient survival. Cases were ...

journal_title：Journal of neuropathology and experimental neurology

authors： Rhodes RH

更新日期：1998-08-01 00:00:00

abstract：:Vascular dementia (VaD) is cognitive decline linked to reduced cerebral blood perfusion, yet there are few therapeutic options to protect cognitive function following cerebrovascular accidents. The purpose of this study was to profile gene expression changes unique to VaD to identify and characterize disease relevant ...

journal_title：Journal of neuropathology and experimental neurology

authors： McKay EC,Beck JS,Khoo SK,Dykema KJ,Cottingham SL,Winn ME,Paulson HL,Lieberman AP,Counts SE

更新日期：2019-05-01 00:00:00

abstract：:The effects of regional heterogeneity on the accuracy of histological grading of gliomas are well known, but little has been reported about its implications for other diagnostic modalities. This study investigated the relationships of regional heterogeneity in tumor proliferative activity, measured by Ki-67 labeling i...

journal_title：Journal of neuropathology and experimental neurology

authors： Coons SW,Johnson PC

更新日期：1993-11-01 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：:Loss of chromosome 1p/19q in oligodendrogliomas represents a powerful predictor of good prognosis. Expression of internexin (INA), a neuronal specific intermediate filament protein, has recently been proposed as a surrogate marker for 1p/19q deletion based on the high degree of correlation between both parameters in o...

journal_title：Journal of neuropathology and experimental neurology

authors： Buckley PG,Alcock L,Heffernan J,Woods J,Brett F,Stallings RL,Farrell MA

更新日期：2011-03-01 00:00:00

abstract：:In situ hybridization (ISH) for JC virus (JCV) is generally applied for the diagnosis of progressive multifocal leukoencephalopathy (PML). To explore the usefulness of immunohistochemistry (IHC) for JCV early proteins, 14 paraffin-embedded postmortem brain specimens with histologic features compatible with PML were te...

journal_title：Journal of neuropathology and experimental neurology

authors： Muñoz-Mármol AM,Mola G,Fernández-Vasalo A,Vela E,Mate JL,Ariza A

更新日期：2004-11-01 00:00:00

abstract：:G-protein coupled receptor kinases (GRKs) constitute a serine/threonine kinase family playing a major role in agonist-induced phosphorylation and desensitization of G-protein coupled receptors. Recently, GRK2 and GRK5 have been demonstrated to phosphorylate alpha-synuclein (Ser129) and other synuclein isoforms. We stu...

journal_title：Journal of neuropathology and experimental neurology

authors： Takahashi M,Uchikado H,Caprotti D,Weidenheim KM,Dickson DW,Ksiezak-Reding H,Pasinetti GM

更新日期：2006-12-01 00:00:00

abstract：:Mild traumatic brain injury resulting from exposure to an explosive blast is associated with significant neurobehavioral outcomes in soldiers. Little is known about the neuropathologic consequences of such an insult to the human brain. This study is an attempt to understand the effects of an explosive blast in a large...

journal_title：Journal of neuropathology and experimental neurology

authors： de Lanerolle NC,Bandak F,Kang D,Li AY,Du F,Swauger P,Parks S,Ling G,Kim JH

更新日期：2011-11-01 00:00:00

abstract：:Intrauterine infection and inflammation have been linked to preterm birth and brain damage. We hypothesized that recombinant human erythropoietin (rhEPO) would ameliorate brain damage in anovine model of fetal inflammation. At 107 +/- 1 day of gestational age (DGA), chronically catheterized fetal sheep received on 3 c...

journal_title：Journal of neuropathology and experimental neurology

authors： Rees S,Hale N,De Matteo R,Cardamone L,Tolcos M,Loeliger M,Mackintosh A,Shields A,Probyn M,Greenwood D,Harding R

更新日期：2010-03-01 00:00:00

abstract：:We investigated the immunohistochemical localization of several proteins related to excitation-contraction coupling and ultrastructural alterations of the sarcotubular system in biopsied muscles from a father and a daughter in a family with permanent myopathy with hypokalemic periodic paralysis (PMPP) due to a mutatio...

journal_title：Journal of neuropathology and experimental neurology

authors： Nagasaka T,Hata T,Shindo K,Adachi Y,Takeuchi M,Saito K,Takiyama Y

更新日期：2020-12-04 00:00:00

abstract：:The main hallmarks of human hippocampal sclerosis are neuronal loss and gliosis; reductions in microvasculature labeling in the cornu Ammonis 1 in this condition have been detected using alkaline phosphatase histochemistry. To determine whether the reduction in alkaline phosphatase activity is coupled with a loss of b...

journal_title：Journal of neuropathology and experimental neurology

authors： Kastanauskaite A,Alonso-Nanclares L,Blazquez-Llorca L,Pastor J,Sola RG,DeFelipe J

更新日期：2009-08-01 00:00:00

abstract：:Morphologic investigations of the human visual pathways have been limited by the infeasibility of modern neuroanatomical approaches. Although contemporary methods for tracing axon pathways (such as tracer injections and electrophysiology) have elucidated the visual system in experimental animals, these techniques cann...

journal_title：Journal of neuropathology and experimental neurology

authors： Sadun AA,Smith LE,Kenyon KR

更新日期：1983-03-01 00:00:00

abstract：:The concern of the potential transmission of animal spongiform encephalopathies to humans, which arose as soon as the interspecies transmission of these diseases was recognized, has been reinforced with the emergence of bovine spongiform encephalopathy (BSE) in cattle. Recent experimental findings suggest that the inf...

journal_title：Journal of neuropathology and experimental neurology

authors： Baron T

更新日期：2002-05-01 00:00:00

abstract：:Genetic and lifestyle-related risk factors for Alzheimer disease (AD) are associated with an increase in oxidative stress, suggesting that oxidative stress is involved at an early stage of the pathologic cascade. Moreover, oxidative stress is mechanistically and chronologically associated with other key features of AD...

journal_title：Journal of neuropathology and experimental neurology

authors： Nunomura A,Castellani RJ,Zhu X,Moreira PI,Perry G,Smith MA

更新日期：2006-07-01 00:00:00

abstract：:Temporal lobe epilepsy (TLE) is characterized by hippocampal sclerosis together with profound losses and phenotypic changes of different classes of interneurons, including those expressing somatostatin (SRIF). To understand the functional significance of the plasticity of SRIF transmission in TLE, unraveling the statu...

journal_title：Journal of neuropathology and experimental neurology

authors： Csaba Z,Pirker S,Lelouvier B,Simon A,Videau C,Epelbaum J,Czech T,Baumgartner C,Sperk G,Dournaud P

更新日期：2005-11-01 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：:Nijmegen breakage syndrome caused by NBS1 germline mutations is a rare autosomal recessive disease with clinical features that include microcephaly, increased radiosensitivity, and predisposition to cancer. NBS1 plays a key role in DNA double-strand break repair and the maintenance of genomic stability. We screened 87...

journal_title：Journal of neuropathology and experimental neurology

authors： Watanabe T,Nobusawa S,Lu S,Huang J,Mittelbronn M,Ohgaki H

更新日期：2009-02-01 00:00:00