A case of Pelizaeus-Merzbacher disease showing increased dosage of the proteolipid protein gene.

abstract：:This report deals with a comparative study on the expression of alpha B crystallin, ubiquitin, stress-response protein 27 (srp 27), srp 72 and phosphorylated neurofilament protein (pNFP) by ballooned neurons in Pick's disease, Creutzfeldt-Jakob disease (CJD), amyotrophic lateral sclerosis (ALS), leptomeningeal carcino...

journal_title：Neuropathology and applied neurobiology

authors： Kato S,Hirano A,Umahara T,Kato M,Herz F,Ohama E

更新日期：1992-08-01 00:00:00

abstract：AIMS:Apolipoprotein A-I (apoA-I), the principal apolipoprotein associated with high-density lipoproteins in the periphery, is also found at high concentrations in the cerebrospinal fluid. Previous studies have reported either no impact or vascular-specific effects of apoA-I knockout (KO) on β-amyloid (Aβ) pathology. Ho...

journal_title：Neuropathology and applied neurobiology

authors： Contu L,Carare RO,Hawkes CA

更新日期：2019-12-01 00:00:00

abstract：:A demyelinating perivascular condition has been observed in the optic nerves of three cats, the lesions of which included the following features: thinly myelinated and demyelinated axons, few normal oligodendrocytes, astroglial scar tissue, lymphocytes, plasma cells, mononuclear phagocytic cells and lipid-laden cells....

journal_title：Neuropathology and applied neurobiology

authors： Cook RD

更新日期：1979-09-01 00:00:00

abstract：:We have previously demonstrated that amyloid beta (Abeta) peptide is acutely toxic to retinal neurones in vivo and that this toxicity is mediated by an indirect mechanism. We have now extended these studies to look at the chronic effect of intravitreal injection of Abeta peptides on retinal ganglion cells (RGC), the p...

journal_title：Neuropathology and applied neurobiology

authors： Walsh DT,Bresciani L,Saunders D,Manca MF,Jen A,Gentleman SM,Jen LS

更新日期：2005-10-01 00:00:00

abstract：:Over the last decade, there has been increasing evidence that hyperactivation of the mechanistic target of rapamycin (mTOR) pathway is a hallmark of malformations of cortical development such as focal cortical dysplasia (FCD) or hemimegalencephaly. The mTOR pathway governs protein and lipid synthesis, cell growth and ...

journal_title：Neuropathology and applied neurobiology

authors： Marsan E,Baulac S

更新日期：2018-02-01 00:00:00

abstract：AIMS:Limb-girdle muscular dystrophy R9 (LGMDR9) is an autosomal recessive disorder caused by mutations in the fukutin-related protein gene (FKRP), encoding a glycosyltransferase involved in α-dystroglycan modification. Muscle atrophy, a significant feature of LGMDR9, occurs by a change in the normal balance between pro...

journal_title：Neuropathology and applied neurobiology

authors： Franekova V,Storjord HI,Leivseth G,Nilssen Ø

更新日期：2020-12-18 00:00:00

abstract：:A detailed investigation of the neuropathology of a horse affected with stringhalt was performed. Qualitative and quantitative light and electron microscopy, and single teased fibre preparations of peripheral nerve demonstrated predominantly axonal degeneration, the stage of which was appropriate for the duration of c...

journal_title：Neuropathology and applied neurobiology

authors： Cahill JI,Goulden BE,Jolly RD

更新日期：1986-09-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a fatal devastating neurodegenerative disorder which predominantly affects the motor neurons in the brain and spinal cord. The death of the motor neurons in ALS causes subsequent muscle atrophy, paralysis and eventual death. Clinical and biological evidence now demonstrates that ...

journal_title：Neuropathology and applied neurobiology

authors： Smethurst P,Sidle KC,Hardy J

更新日期：2015-08-01 00:00:00

abstract：:The development of granule cells and the formation of synapses with mossy fibres were investigated in cerebella from jaundiced (j/j) Gunn rats with hereditary hyperbilirubinemia. The external granular layer was thinner than that in cerebella from heterozygous (+/j) Gunn rats without hyperbilirubinemia on days 12 and 1...

journal_title：Neuropathology and applied neurobiology

authors： Takagishi Y,Yamamura H

更新日期：1993-08-01 00:00:00

abstract：:The cellular distribution of the lysosomal proteinase cathepsin D was studied in a series of 76 neoplasms and 18 non-neoplastic tissues from the human central nervous system, using a well-characterized polyclonal antibody in a peroxidase-antiperoxidase technique. In the normal and developing brain, cathepsin D is conf...

journal_title：Neuropathology and applied neurobiology

authors： Robson DK,Ironside JW,Reid WA,Bogue PR

更新日期：1990-02-01 00:00:00

abstract：:Apoptotic bodies are frequently found in oligodendrogliomas, particularly in the anaplastic subtype. A range of proteins, such as those of the Bcl family, are implicated in the control of apoptosis. The ratio of antagonists of apoptosis, such as Bcl-2, to agonists, such as Bax, is thought to determine the outcome for ...

journal_title：Neuropathology and applied neurobiology

authors： Delgado MB,Anderson JR,Whittle IR,Wharton SB

更新日期：1999-10-01 00:00:00

abstract：:Minute amounts of tetanus toxin injected into the hippocampus of rats results in an epileptiform syndrome. When the toxin injection is made unilaterally or bilaterally into the ventral hippocampus, about one-third of animals with seizures show bilateral neuronal loss in dorsal CA1 of the hippocampus after 1 week. In a...

journal_title：Neuropathology and applied neurobiology

authors： Shaw JA,Perry VH,Mellanby J

更新日期：1994-08-01 00:00:00

abstract：:Human tumour xenografts maintained in nude mice are a valuable research tool. The passaging and maintenance of human tumour xenografts in immune-deficient animals are expensive and labour-intensive. This study presents a protocol that permits long-term cryopreservation of viable glioblastoma xenograft tissue pieces in...

journal_title：Neuropathology and applied neurobiology

authors： Goike HM,Asplund AC,Pettersson EH,Liu L,Ichimura K,Collins VP

更新日期：2000-04-01 00:00:00

abstract：AIMS:We observed a microvascular structure in the cerebral cortex that has not, to our knowledge, been previously described. We have termed the structure a 'raspberry', referring to its appearance under a bright-field microscope. We hypothesized that raspberries form through angiogenesis due to some form of brain ischa...

journal_title：Neuropathology and applied neurobiology

authors： Ek Olofsson H,Englund E

更新日期：2019-10-01 00:00:00

abstract：:The spectrum of glioneuronal lesions underlying intractable epilepsies includes malformative pathologies like focal cortical dysplasia (FCD); and neoplastic lesions like gangliogliomas (GG) and dysembryoplastic neuroepithelial tumours (DNT). These may occur either singly or as dual lesions, having simultaneous presenc...

journal_title：Neuropathology and applied neurobiology

authors： Deb P,Sharma MC,Tripathi M,Sarat Chandra P,Gupta A,Sarkar C

更新日期：2006-10-01 00:00:00

abstract：AIMS:The deposition of amyloid peptides (A beta) in the cortex and hippocampus is the primary trigger of Alzheimer's disease (AD). Recent studies also indicated that the M2 subtype of muscarinic acetylcholine receptors (M2mAChR) may be a key molecule involved in cognitive dysfunction. Thus, the purpose of this study wa...

journal_title：Neuropathology and applied neurobiology

authors： González I,Arévalo-Serrano J,Pérez JL,Gonzalo P,Gonzalo-Ruiz A

更新日期：2008-10-01 00:00:00

abstract：AIMS:Transition metals, oxidative stress and neuroinflammation have been proposed as part of a vicious cycle in central nervous system neurodegeneration. Our aim was to study the anti-inflammatory effect of pioglitazone, a peroxisome proliferative activated receptor-γ agonist, on iron-induced oxidative injury in rat br...

journal_title：Neuropathology and applied neurobiology

authors： Yu HC,Feng SF,Chao PL,Lin AM

更新日期：2010-12-01 00:00:00

abstract：AIMS:Normal neurovascular coupling, mediated by the fine interplay and communication of cells within the neurovascular unit, is critical for maintaining normal brain activity and cognitive function. This study investigated whether, with advancing age there is disruption of neurovascular coupling and specific cellular c...

journal_title：Neuropathology and applied neurobiology

authors： Duncombe J,Lennen RJ,Jansen MA,Marshall I,Wardlaw JM,Horsburgh K

更新日期：2017-10-01 00:00:00

abstract：AIMS:Frontotemporal lobar degeneration (FTLD) and motor neurone disease are linked by the possession of a hexanucleotide repeat expansion in C9ORF72, and both show neuronal cytoplasmic inclusions within cerebellar and hippocampal neurones which are TDP-43 negative but immunoreactive for p62 and dipeptide repeat protein...

journal_title：Neuropathology and applied neurobiology

authors： Baborie A,Griffiths TD,Jaros E,Perry R,McKeith IG,Burn DJ,Masuda-Suzukake M,Hasegawa M,Rollinson S,Pickering-Brown S,Robinson AC,Davidson YS,Mann DM

更新日期：2015-08-01 00:00:00

abstract：AIMS:To explore alterations in the expression of genes encoding enzymes involved in purine metabolism in Parkinson's disease (PD) brains as purines are the core of the DNA, RNA, nucleosides and nucleotides which participate in a wide variety of crucial metabolic pathways. METHODS:Analysis of mRNA using real-time quant...

journal_title：Neuropathology and applied neurobiology

authors： Garcia-Esparcia P,Hernández-Ortega K,Ansoleaga B,Carmona M,Ferrer I

更新日期：2015-12-01 00:00:00

abstract：AIMS:Resident and peripherally derived glioma associated microglia/macrophages (GAMM) play a key role in driving tumour progression, angiogenesis, invasion and attenuating host immune responses. Differentiating these cells' origins is challenging and current preclinical models such as irradiation-based adoptive transfe...

journal_title：Neuropathology and applied neurobiology

authors： Yu K,Youshani AS,Wilkinson FL,O'Leary C,Cook P,Laaniste L,Liao A,Mosses D,Waugh C,Shorrock H,Pathmanaban O,Macdonald A,Kamaly-Asl I,Roncaroli F,Bigger BW

更新日期：2019-02-01 00:00:00

abstract：:A 50-year-old female patient died of an untreatable glioma apoplecticum. At autopsy a strongly vascularized glial tumour was found. The criteria for malignancy according to the WHO classification were only partially fulfilled by this tumour which displayed morphological features of an astrocytoma but could not be furt...

journal_title：Neuropathology and applied neurobiology

authors： Radner H,Kleinert R,Vennigerholz F,Denk H

更新日期：1990-04-01 00:00:00

abstract：AIMS:14,15-Epoxyeicosatrienoic acid (14,15-EET) is abundantly expressed in brain and exerts protective effects against ischaemia. 14,15-EET is hydrolysed by soluble epoxide hydrolase (sEH). sEH-/- mice show a higher level of 14,15-EET in the brain. Astrocytes play a pivotal role in neuronal survival under ischaemic con...

journal_title：Neuropathology and applied neurobiology

authors： Yuan L,Liu J,Dong R,Zhu J,Tao C,Zheng R,Zhu S

更新日期：2016-12-01 00:00:00

abstract：AIM:Levels of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) are robustly increased in spinal muscular atrophy (SMA) patient fibroblasts and mouse models. We therefore wanted to establish whether changes in UCHL1 contribute directly to disease pathogenesis, and to assess whether pharmacological inhibition of UCHL1 re...

journal_title：Neuropathology and applied neurobiology

authors： Powis RA,Mutsaers CA,Wishart TM,Hunter G,Wirth B,Gillingwater TH

更新日期：2014-12-01 00:00:00

abstract：:A single dose (19 mg kg-1) of triethyllead given to weanling rats produces necrosis in a small number of hippocampal pyramidal (CA3) and hilar neurons with reversible changes in the remaining neurons of this region. The sequence of events has been studied by light and electron microscopy over a period from 12 h to 14 ...

journal_title：Neuropathology and applied neurobiology

authors： Nolan CC,Brown AW

更新日期：1989-09-01 00:00:00

abstract：AIMS:Multiple sclerosis (MS) and neuromyelitis optica (NMO) are inflammatory autoimmune diseases that affect the central nervous system. Several genome-wide and candidate gene studies have identified genetic polymorphisms associated with the risk of MS or NMO. In particular, two recently published studies of meta-analy...

journal_title：Neuropathology and applied neurobiology

authors： Park TJ,Kim HJ,Kim JH,Bae JS,Cheong HS,Park BL,Shin HD

更新日期：2013-08-01 00:00:00

abstract：:The anatomical arrangement of the pia mater suggests that it may act as a regulatory interface between cerebrospinal fluid and the surface of the brain and between arterioles within the brain and the surrounding neural tissue. However, the functional aspects of such a barrier are difficult to evaluate in vivo. In the ...

journal_title：Neuropathology and applied neurobiology

authors： Feurer DJ,Weller RO

更新日期：1991-10-01 00:00:00

abstract：:The epidermal growth factor receptor (EGFR) is a protooncogene that is frequently observed with alterations in late stage gliomas, suggesting an important role of this gene in glial tumorigenesis and progression. In this study we evaluated an antisense EGFR approach as an alternative therapeutic modality for glioblast...

journal_title：Neuropathology and applied neurobiology

authors： Tian XX,Lam PY,Chen J,Pang JC,To SS,Di-Tomaso E,Ng HK

更新日期：1998-10-01 00:00:00

abstract：AIMS:This study explores the neuroprotective effects and mechanisms of N-acetyl-L-cysteine (NAC) in mice exposed to cadmium (Cd). METHODS:NAC (150 mg/kg) was intraperitoneally administered to mice exposed to Cd (10-50 mg/L) in drinking water for 6 weeks. The changes of cell damage and death, reactive oxygen species (R...

journal_title：Neuropathology and applied neurobiology

authors： Chen S,Ren Q,Zhang J,Ye Y,Zhang Z,Xu Y,Guo M,Ji H,Xu C,Gu C,Gao W,Huang S,Chen L

更新日期：2014-10-01 00:00:00

abstract：:Adhalin and alpha-dystroglycan are two components of a complex of proteins that, in conjunction with dystrophin, provide a link between the subsarcolemmal cytoskeleton and the basal lamina of the extracellular matrix of skeletal muscle. In the absence of dystrophin, in Duchenne muscular dystrophy (DMD) and the mdx mou...

journal_title：Neuropathology and applied neurobiology

authors： Cullen MJ,Walsh J,Roberds SL,Campbell KP

更新日期：1996-02-01 00:00:00