Increased levels of UCHL1 are a compensatory response to disrupted ubiquitin homeostasis in spinal muscular atrophy and do not represent a viable therapeutic target.

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：:The human chromosome 22 displays an unusual concentration of medically relevant genes, especially gene(s) involved in malignancies of the central nervous system. A full genomic and transcriptional characterization of this autosome will no doubt make a strong impact on the understanding of some of the processes which l...

journal_title：Neuropathology and applied neurobiology

authors： Dumanski JP

更新日期：1996-10-01 00:00:00

abstract：:We have examined the influence that axons may have on the expression of proteolipid protein (PLP), the major myelin protein of the CNS. Partial transections were made of the optic nerve of adult rats to produce approximately a 50% loss of axons. Twenty-eight days after lesioning, sections of the distal nerves were imm...

journal_title：Neuropathology and applied neurobiology

authors： McPhilemy K,Griffiths IR,Mitchell LS,Kennedy PG

更新日期：1991-08-01 00:00:00

abstract：:Axonal damage in multiple sclerosis (MS) is correlated to disease progression. Early axonal damage may be compensated for by regenerative processes. Growth-associated protein 43 (GAP-43) is a marker for axonal growth and synaptogenesis in various neurodegenerative diseases. We investigated the expression of GAP-43 in ...

journal_title：Neuropathology and applied neurobiology

authors： Teunissen CE,Dijkstra CD,Jasperse B,Barkhof F,Vanderstichele H,Vanmechelen E,Polman CH,Bö L

更新日期：2006-06-01 00:00:00

abstract：:Total protein, alphafetoprotein, albumin, prealbumin, alpha-1-antitrypsin, transferrin and ceruloplasmin levels were measured in samples of human fetal and neonatal cerebrospinal fluid (CSF) (97 cases), obtained between 12 and 41 weeks of gestation. In 39 of these cases (13 to 40 weeks gestation) plasma was also avail...

journal_title：Neuropathology and applied neurobiology

authors： Bell JE,Fryer AA,Collins M,Marshall T,Jones PW,Strange R,Hume R

更新日期：1991-12-01 00:00:00

abstract：:The expression of two heat shock proteins, HSP72 and p57, in addition to ubiquitin, has been studied immunocytochemically in nine amyotrophic lateral sclerosis (ALS) cases and 10 age-matched controls. HSP72 and p57 antibodies did not identify the characteristic ubiquitin-immunoreactive inclusions present in anterior h...

journal_title：Neuropathology and applied neurobiology

authors： Garofalo O,Kennedy PG,Swash M,Martin JE,Luthert P,Anderton BH,Leigh PN

更新日期：1991-02-01 00:00:00

abstract：:The avirulent demyelinating strain A7(74) of Semliki Forest virus after passage through mouse brain in vivo and mouse brain cell cultures has been shown to react immunologically with immune sera against galactocerebroside, glucocerebroside, total ganglioside and GT1b ganglioside but not against myelin or sulphatide . ...

journal_title：Neuropathology and applied neurobiology

authors： Webb HE,Mehta S,Gregson NA,Leibowitz S

更新日期：1984-03-01 00:00:00

abstract：:Changes of 5'-nucleotidase (5'-N) activity during subcultivation of C6 clonal rat glioma cells were studied histochemically, cytochemically and biochemically. Two different cell populations were seeded with high or low cell density respectively. 5'-N activity in cultured cells increased continuously in both population...

journal_title：Neuropathology and applied neurobiology

authors： Amano S,Sasahara M,Yamada E,Kataoka H,Hazama F

更新日期：1985-05-01 00:00:00

abstract：:The four GPI-anchored cell adhesion molecules that exemplify the IgLON family are most highly expressed in the nervous system and associate to form up to six different heterodimeric 'Diglons' that can modify cell adhesion and inhibit axon migration. Recently, two members, OPCML and LSAMP, were identified as putative t...

journal_title：Neuropathology and applied neurobiology

authors： Reed JE,Dunn JR,du Plessis DG,Shaw EJ,Reeves P,Gee AL,Warnke PC,Sellar GC,Moss DJ,Walker C

更新日期：2007-02-01 00:00:00

abstract：BACKGROUND:Juvenile neuronal ceroid lipofuscinoses (JNCL) or juvenile Batten disease is a recessively inherited childhood neurodegenerative disorder resulting from a mutation in CLN3, which encodes a putative lysosomal protein of unknown function. AIM:Recent evidence suggests that a disruption in CLN3 function results...

journal_title：Neuropathology and applied neurobiology

authors： Chan CH,Ramirez-Montealegre D,Pearce DA

更新日期：2009-04-01 00:00:00

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：:Ultrastructural studies of spongiform encephalopathy (SE) reveal no very early pathological changes in kuru where membrane lamellation has been reported. This observation is challenged. In the later stages of SE, two main theories are examined--the spiroplasma theory and the prion (6 nm filament) theory. Neither are s...

journal_title：Neuropathology and applied neurobiology

authors： Gray EG

更新日期：1986-03-01 00:00:00

abstract：:Serial sections of the brains of two cases with Alzheimer's disease were stained with the standard Bodian, modified Bielschowsky (reformed Gros-Schultze's modification) and thioflavin S methods. The numbers of demonstrated Alzheimer's neurofibrillary tangles (NFTs) were different between the two silver stains: from 15...

journal_title：Neuropathology and applied neurobiology

authors： Yamamoto T,Hirano A

更新日期：1986-01-01 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：INTRODUCTION:Magnetic resonance imaging (MRI) cerebral microbleeds (CMB) arise from ferromagnetic haemosiderin iron assumed to derive from extravasation of erythrocytes. Light microscopy of ageing brain frequently reveals foci of haemosiderin from single crystalloids to larger, predominantly perivascular, aggregates. T...

journal_title：Neuropathology and applied neurobiology

authors： Janaway BM,Simpson JE,Hoggard N,Highley JR,Forster G,Drew D,Gebril OH,Matthews FE,Brayne C,Wharton SB,Ince PG,MRC Cognitive Function and Ageing Neuropathology Study.

更新日期：2014-04-01 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：:A disorder of the gracile axonal dystrophy (GAD) mutant mouse is characterized by a neuromuscular disease with sensory ataxia (detectable 30 days after birth) and paresis of the hindlimbs (detectable at 80 days). In the sensory ataxia stage, histological study of the primary sensory system shows that, in addition to t...

journal_title：Neuropathology and applied neurobiology

authors： Oda K,Yamazaki K,Miura H,Shibasaki H,Kikuchi T

更新日期：1992-06-01 00:00:00

abstract：AIMS:Granulovacuolar degeneration involves the accumulation of large, double membrane-bound bodies within certain neurones during the course of Alzheimer's disease (AD) and other adult-onset dementias. Because of the two-layer membrane morphology, it has been proposed that the bodies are related to autophagic organelle...

journal_title：Neuropathology and applied neurobiology

authors： Funk KE,Mrak RE,Kuret J

更新日期：2011-04-01 00:00:00

abstract：:Accumulating evidence indicates an important pathophysiological role of brain inflammation in epilepsy. In this review, we will provide an update of specific inflammatory pathways that have been proposed to be crucial in the underlying molecular mechanisms of epilepsy, including the interleukin-1 receptor/toll-like re...

journal_title：Neuropathology and applied neurobiology

authors： van Vliet EA,Aronica E,Vezzani A,Ravizza T

更新日期：2018-02-01 00:00:00

abstract：:Two HIV-positive male patients presented with a variety of symptoms including hemiparesis, unsteadiness, progressive loss of vision and poor memory. There were multiple non-enhancing lesions shown by CT scan in the white matter of the cerebral hemispheres. Specimens obtained by burr-hole biopsy showed the features of ...

journal_title：Neuropathology and applied neurobiology

authors： Scaravilli F,Ellis DS,Tovey G,Harcourt-Webster JN,Guiloff RJ,Sinclair E

更新日期：1989-09-01 00:00:00

abstract：AIMS:Amyloid-β (Aβ) oligomers trigger synaptic degeneration that precedes plaque and tangle pathology. However, the signalling molecules that link Aβ oligomers to synaptic pathology remain unclear. Here, we addressed the potential role of RAPGEF2 as a novel signalling molecule in Aβ oligomer-induced synaptic and cognit...

journal_title：Neuropathology and applied neurobiology

authors： Jang YN,Jang H,Kim GH,Noh JE,Chang KA,Lee KJ

更新日期：2020-12-20 00:00:00

abstract：:Multiple system atrophy (MSA) is an unrelenting, sporadic, adult-onset, neurodegenerative disease of unknown aetiology. Its clinically progressive course is characterized by a variable combination of parkinsonism, cerebellar ataxia and/or autonomic dysfunction. Neuropathological examination often reveals gross abnorma...

journal_title：Neuropathology and applied neurobiology

authors： Ahmed Z,Asi YT,Sailer A,Lees AJ,Houlden H,Revesz T,Holton JL

更新日期：2012-02-01 00:00:00

abstract：AIMS:Adult neurogenesis is well described in the subventricular zone of the lateral ventricle walls and in the subgranular zone of the hippocampal dentate gyrus. However, recent studies indicate that self-renewal of neural stem cells (NSCs) is not restricted to these niches, but that diverse areas of the adult brain ar...

journal_title：Neuropathology and applied neurobiology

authors： Sanin V,Heeß C,Kretzschmar HA,Schüller U

更新日期：2013-08-01 00:00:00

abstract：:Tubular vesicular and net-like dissolution of myelin sheaths associated with complete demyelination and preservation of axons, is described in the brain, obtained within 4 h of death, from a patient who died with acute multiple sclerosis (MS). It was rare, being found in only three out of twenty-three blocks examined,...

journal_title：Neuropathology and applied neurobiology

authors： Kirk J

更新日期：1979-08-01 00:00:00

abstract：:Obstructive hydrocephalus was induced in 12-day-old rats by the infusion of kaolin into the cisterna magna. After 7 days, cortical and ependymal lesions were made with thin wire in non-hydrocephalic brains and in hydrocephalic brains. The wounds were allowed to heal for times ranging from 1 day to 10 days post-lesion,...

journal_title：Neuropathology and applied neurobiology

authors： Collins P,Goulding DA

更新日期：1992-08-01 00:00:00

abstract：:Using a combined biochemical and histological approach certain conclusions can be drawn as to the origin of the increase in lysosomal enzymes in white matter from MS brains. Firstly, there is a gradient of lysosomal enzyme activity, plaque greater than periplaque greater than macroscopically normal white matter, which...

journal_title：Neuropathology and applied neurobiology

authors： McKeown SR,Allen IV

更新日期：1978-11-01 00:00:00

abstract：:Sandhoff disease is a heritable lysosomal storage disease resulting from impaired degradation of GM2 ganglioside and related substrates. A mouse model of Sandhoff disease created by gene targeting displays progressive neurological manifestations, similar to patients with the disease. In the present in vivo and in vitr...

journal_title：Neuropathology and applied neurobiology

authors： Sango K,Yamanaka S,Ajiki K,Tokashiki A,Watabe K

更新日期：2002-02-01 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：: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：AIM:Pilocytic astrocytomas represent the most common paediatric tumours of the central nervous system. Dissemination through the ventricular system occurs rarely in patients with pilocytic astrocytomas; however, it is more common in infants with diencephalic tumours, and is associated with a poor outcome. Despite histo...

journal_title：Neuropathology and applied neurobiology

authors： Gessi M,Engels AC,Lambert S,Rothämel T,von Hornstein S,Collins VP,Denkhaus D,Gnekow A,Pietsch T

更新日期：2016-04-01 00:00:00