The neuropathology, pathophysiology and genetics of multiple system atrophy.

abstract：INTRODUCTION:Cerebral ischaemia is the defining pathophysiological abnormality in most forms of vascular dementia (VAD), but the pathogenesis of the dementia remains poorly understood. In Alzheimer's disease (AD), there is early loss of synaptic proteins, but these have been little studied in VAD. MATERIALS AND METHOD...

journal_title：Neuropathology and applied neurobiology

authors： Sinclair LI,Tayler HM,Love S

更新日期：2015-06-01 00:00:00

abstract：AIMS:Rodents exposed to 3,3'-iminodipropionitrile (IDPN) develop an axonopathy similar to that observed in amyotrophic lateral sclerosis motor neurones, in which neurofilaments accumulate in swollen proximal axon segments. This study addressed the hypotheses that this proximal axonopathy is associated with loss of neur...

journal_title：Neuropathology and applied neurobiology

authors： Soler-Martín C,Vilardosa U,Saldaña-Ruíz S,Garcia N,Llorens J

更新日期：2012-02-01 00:00:00

abstract：:Semliki Forest virus infections in mice produce an encephalitis with demyelination. If before giving the virus the mice are treated with muramyl dipeptide in Freund's incomplete adjuvant, there is a significant increase in demyelination. If ovalbumin is added to the above and then followed after an interval by a secon...

journal_title：Neuropathology and applied neurobiology

authors： Illavia SJ,Webb HE,Colover J

更新日期：1988-11-01 00:00:00

abstract：:The pathological findings in Alzheimer's disease (AD) are partly attributed to alterations in calcium-binding protein (CBP) functions. We showed previously that immunoreactivity of secretagogin, a recently cloned CBP, in the human hippocampus is restricted to pyramidal neurones and that the amount of immunoreactive ne...

journal_title：Neuropathology and applied neurobiology

authors： Attems J,Preusser M,Grosinger-Quass M,Wagner L,Lintner F,Jellinger K

更新日期：2008-02-01 00:00:00

abstract：AIM:To understand the progression of CLN1 disease and develop effective therapies we need to characterize early sites of pathology. Therefore, we performed a comprehensive evaluation of the nature and timing of early CLN1 disease pathology in the spinal cord, which appears especially vulnerable, and how this may affect...

journal_title：Neuropathology and applied neurobiology

authors： Nelvagal HR,Dearborn JT,Ostergaard JR,Sands MS,Cooper JD

更新日期：2020-08-25 00:00:00

abstract：:Mitochondrial dysfunction leads to oxygen free radical (ROS) generation with consequent oxidative stress and cellular damage. Recently, activation of the cellular antioxidant system and apoptosis were demonstrated in skeletal muscle fibres from patients with mitochondrial diseases, but the underlying mechanisms remain...

journal_title：Neuropathology and applied neurobiology

authors： Filosto M,Tonin P,Vattemi G,Savio C,Rizzuto N,Tomelleri G

更新日期：2003-02-01 00:00:00

abstract：:Over the past few years, the traditional view of brain tumorigenesis has been revolutionized by advances in genomic medicine, molecular biology, stem cell biology and genetically engineered small-animal modelling. We now appreciate that paediatric brain tumours arise following specific genetic mutations in specialized...

journal_title：Neuropathology and applied neurobiology

authors： Thangarajh M,Gutmann DH

更新日期：2012-06-01 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：AIM:Borna disease virus (BDV) induces a persistent infection in the central nervous system (CNS) accompanied by a non-purulent meningoencephalitis. BDV-infection of Lewis rats provides an important model to investigate basic principles of neurotropism, viral persistence and resulting dysfunctions. To date, the in vivo ...

journal_title：Neuropathology and applied neurobiology

authors： Werner-Keiss N,Garten W,Richt JA,Porombka D,Algermissen D,Herzog S,Baumgärtner W,Herden C

更新日期：2008-12-01 00:00:00

abstract：:Vascular endothelial growth factor (VEGF) has recently been implicated in several neurological disorders. Apart from its prominent role in angiogenesis, VEGF has been shown to have direct effects on neuronal and glial cells through activation of different VEGF receptor (VEGFR) types. In the present study the expressio...

journal_title：Neuropathology and applied neurobiology

authors： Spliet WG,Aronica E,Ramkema M,Witmer AN,Schlingemann RO,de Jong JM,Troost D

更新日期：2004-08-01 00:00:00

abstract：:Mitochondrial abnormalities, in particular the accumulation of mitochondrial DNA mutations, have been proposed as a potential cause of normal ageing. One group of patients with mtDNA disorders have a nuclear DNA defect which accelerates the chronological accumulation of mitochondrial DNA mutations. These patients prov...

journal_title：Neuropathology and applied neurobiology

authors： Cottrell DA,Ince PG,Wardell TM,Turnbull DM,Johnson MA

更新日期：2001-06-01 00:00:00

abstract：:Two cases are described, one with a multifocal cranial and limb neuropathy of adult onset associated with optic neuropathy, and the other with a diffuse demyelinating neuropathy characterized by congenital cataract, mental retardation and progressive lower limb paresis with an onset in childhood. Extensive investigati...

journal_title：Neuropathology and applied neurobiology

authors： Thomas PK,King RH,Workman JM,Schröder JM

更新日期：2000-12-01 00:00:00

abstract：:Protein aggregation, mitochondrial impairment and oxidative stress are common to multiple neurodegenerative diseases. Homeostasis is regulated by a balanced set of anabolic and catabolic responses, which govern removal and repair of damaged proteins and organelles. Macroautophagy is an evolutionarily conserved pathway...

journal_title：Neuropathology and applied neurobiology

authors： Cherra SJ 3rd,Dagda RK,Chu CT

更新日期：2010-04-01 00:00:00

abstract：:The mechanism of the neuroprotective effect of hyperbaric oxygenation remains unclear although its clinical benefits have been well recognized for human ischaemic neuronal disease. The preventive effect of hyperbaric oxygenation against delayed neuronal death was investigated in the gerbil following transient forebrai...

journal_title：Neuropathology and applied neurobiology

authors： Konda A,Baba S,Iwaki T,Harai H,Koga H,Kimura T,Takamatsu J

更新日期：1996-08-01 00:00:00

abstract：:Ciliary neurotrophic factor (CNTF) may be implicated in the pathogenetic mechanisms of hepatic encephalopathy. We tested this hypothesis by treating confluent primary cultures of rat astroglial cells with ammonium chloride for various periods and analysing the effect of ammonia on the signalling pathway that regulates...

journal_title：Neuropathology and applied neurobiology

authors： Bodega G,Suárez I,Almonacid L,Ciordia S,Beloso A,López-Fernández LA,Zaballos A,Fernández B

更新日期：2007-08-01 00:00:00

abstract：AIMS:Brain mural cells (BMC), smooth muscle cells and pericytes, interact closely with endothelial cells and modulate numerous cerebrovascular functions. A loss of BMC function is suspected to play a role in the pathophysiology of Alzheimer's Disease (AD). METHODS:BMC markers, namely smooth muscle alpha actin (α-SMA) ...

journal_title：Neuropathology and applied neurobiology

authors： Bourassa P,Tremblay C,Schneider JA,Bennett DA,Calon F

更新日期：2020-08-01 00:00:00

abstract：:Cyclins are important regulators of the cell cycle; there is increasing evidence that some cyclins are positively involved in carcinogenesis. Amplification and translocation of the cyclin genes and overexpression of their mRNAs and proteins have been observed in a variety of tumours. We studied cyclin A protein in ast...

journal_title：Neuropathology and applied neurobiology

authors： Chakrabarty A,Bridges LR

更新日期：1998-06-01 00:00:00

abstract：:The nature of the glial and inflammatory cell responses to infection in scrapie-affected brains was studied in terminally-affected mice of five scrapie models. There were marked astrocytic and microglial responses. Microglia showed increased staining of the surface antigens F4/80, leucocyte-common antigen, type 3 comp...

journal_title：Neuropathology and applied neurobiology

authors： Williams AE,Lawson LJ,Perry VH,Fraser H

更新日期：1994-02-01 00:00:00

abstract：AIMS:Oxidative damage and an associated DNA damage response (DDR) are evident in mild cognitive impairment and early Alzheimer's disease, suggesting that neuronal dysfunction resulting from oxidative DNA damage may account for some of the cognitive impairment not fully explained by Alzheimer-type pathology. METHODS:Fr...

journal_title：Neuropathology and applied neurobiology

authors： Simpson JE,Ince PG,Minett T,Matthews FE,Heath PR,Shaw PJ,Goodall E,Garwood CJ,Ratcliffe LE,Brayne C,Rattray M,Wharton SB,MRC Cognitive Function and Ageing Neuropathology Study Group.

更新日期：2016-02-01 00:00:00

abstract：:Mutations in the tau gene cause familial frontotemporal dementia with parkinsonism linked to chromosome 17 characterized by filamentous tau protein deposits. Here we describe the clinical and neuropathological features of a case from a newly identified family with an intron 10+3-splice site mutation in the tau gene. T...

journal_title：Neuropathology and applied neurobiology

authors： Neumann M,Mittelbronn M,Simon P,Vanmassenhove B,de Silva R,Lees A,Klapp J,Meyermann R,Kretzschmar HA

更新日期：2005-08-01 00:00:00

abstract：:In a stereological study of the human substantia nigra (SN), the total number of melanin-positive and melanin-negative neurones from 28 male subjects aged 19-92 years was estimated using a uniform sampling design and optical disectors. There was a significant decrease in the total number of melanin-positive neurones a...

journal_title：Neuropathology and applied neurobiology

authors： Cabello CR,Thune JJ,Pakkenberg H,Pakkenberg B

更新日期：2002-08-01 00:00:00

abstract：AIMS:Insufficient oligodendroglial differentiation of oligodendroglial progenitor cells (OPCs) is suggested to be responsible for remyelination failure and astroglial scar formation in Theiler's murine encephalomyelitis (TME). The aim of the present study is to identify molecular key regulators of OPC differentiation i...

journal_title：Neuropathology and applied neurobiology

authors： Sun Y,Lehmbecker A,Kalkuhl A,Deschl U,Sun W,Rohn K,Tzvetanova ID,Nave KA,Baumgärtner W,Ulrich R

更新日期：2015-04-01 00:00:00

abstract：:An unusual inherited progressive distal myopathy of early childhood onset is described in two sisters from a consanguineous Asian family. Motor milestones were normal but gait deteriorated slowly thereafter with development of generalized hypotonia and muscle weakness particularly in the wrist extensors and hand muscl...

journal_title：Neuropathology and applied neurobiology

authors： Mahon M,Kristmundsdottir F,Cumming WJ,Noronha MJ

更新日期：1989-01-01 00:00:00

abstract：AIMS:The serotonin 2A receptor (HTR2A) is widely expressed in the brain and involved in the modulation of fear, mood, anxiety and other symptoms. HTR2A and HTR2A gene variations are implicated in depression, schizophrenia, anxiety and obsessive-compulsive disorder. To understand HTR2A signalling changes in psychiatric ...

journal_title：Neuropathology and applied neurobiology

authors： Marinova Z,Monoranu CM,Fetz S,Walitza S,Grünblatt E

更新日期：2015-06-01 00:00:00

abstract：:Defects of mitochondrial function have been proposed as a potential mechanism in the development and pathogenesis of Alzheimer's disease (AD) and neuronal apoptosis. Mitochondrial enzyme-deficient pyramidal neurones are found in greater quantities in the hippocampus of AD patients than in age-matched controls. The pre...

journal_title：Neuropathology and applied neurobiology

authors： Cottrell DA,Borthwick GM,Johnson MA,Ince PG,Turnbull DM

更新日期：2002-10-01 00:00:00

abstract：:Mutation of the LRRK2 gene has been associated with autosomal dominant parkinsonism. An R1441C pathogenic substitution was identified in Family D, a large Western Nebraskan kindred, with four members demonstrating pleomorphic pathology at autopsy. One member of this family displayed tau pathology suggestive of progres...

journal_title：Neuropathology and applied neurobiology

authors： Ross OA,Whittle AJ,Cobb SA,Hulihan MM,Lincoln SJ,Toft M,Farrer MJ,Dickson DW

更新日期：2006-02-01 00:00:00

abstract：:Inflammatory cells from the meninges of guinea-pigs with acute EAE have been isolated and quantitated. A mean of 7.5 x 10(6) cells were recovered from the brains of animals showing clinical signs of disease in contrast to only 1.5 x 10(6) cells in the controls. The immunological specificity of the meningeal inflammato...

journal_title：Neuropathology and applied neurobiology

authors： Allsopp G,Parker D,Hinrichs DJ,Turk JL

更新日期：1981-03-01 00:00:00

abstract：:Focal cortical dysplasia (FCD), which is often associated with intractable epilepsy, is a form of abnormal structure of the cerebral cortex caused by a disorder of normal neocortical development. In such cerebral lesions obtained from four patients (two male, two female; average age 32.3 years at operation), the immun...

journal_title：Neuropathology and applied neurobiology

authors： Nishio S,Morioka T,Hamada Y,Hisada K,Fukui M

更新日期：1999-06-01 00:00:00

abstract：:A quantitative assessment was made of the myogenic capability in vitro of muscle cells from dystrophic (129 Rej dy/dy) and normal mice from birth to 5 months old. Seeding efficiency was increased in dystrophic cells from neonatal and 1-week-old mice compared to age-matched controls. The extent of myogenesis in culture...

journal_title：Neuropathology and applied neurobiology

authors： Summers PJ,Parsons R

更新日期：1981-07-01 00:00:00

abstract：:Irradiation of a rat hind limb at 15 days of age caused marked shortening of the limb, with a consequent reduction in nerve length. The tibial nerves of the irradiated limbs were examined qualitatively and quantitatively, and compared with the unirradiated nerve. There was no evidence of fibre degeneration and only ve...

journal_title：Neuropathology and applied neurobiology

authors： Jacobs JM,Myers R

更新日期：1993-02-01 00:00:00