Protein homeostasis in LGMDR9 (LGMD2I) - The role of ubiquitin-proteasome and autophagy-lysosomal system.

abstract：:Dementia with Lewy bodies (DLB) is characterized by the widespread presence of Lewy bodies (LBs) in the brain. alpha-Synuclein, the main component of LBs, is expressed as two main isoforms (112 and 140), but little is known about their differential expression in the brain. We compared alpha-synuclein 112 and alpha-syn...

journal_title：Neuropathology and applied neurobiology

authors： Beyer K,Lao JI,Carrato C,Mate JL,López D,Ferrer I,Ariza A

更新日期：2004-12-01 00:00:00

abstract：:Clinicopathological observations suggest there is considerable overlap between vascular dementia (VaD) and Alzheimer's disease (AD). We used immunochemical methods to compare quantities of amyloid-beta (Abeta) peptides in post mortem brain samples from VaD, AD subjects and nondemented ageing controls. Total Abeta pept...

journal_title：Neuropathology and applied neurobiology

authors： Lewis H,Beher D,Cookson N,Oakley A,Piggott M,Morris CM,Jaros E,Perry R,Ince P,Kenny RA,Ballard CG,Shearman MS,Kalaria RN

更新日期：2006-04-01 00:00:00

abstract：:Ubiquitin targets proteins for attack by certain proteolytic enzymes, but the ubiquitinated cytoplasmic inclusions seen in some chronic neurodegenerative diseases may indicate the occurrence of reparative rather than destructive metabolic events. We have examined the production of ubiquitin in motor neurons of the rat...

journal_title：Neuropathology and applied neurobiology

authors： Savedia S,Kiernan JA

更新日期：1994-12-01 00:00:00

abstract：:An elderly man suffering from dementia associated with hypertension developed right-sided parkinsonism marked by rigidity and flexion. At autopsy the brain showed generalized vascular changes chiefly in the white matter, diffuse plaque and neurofibrillary tangle formation in the temporal cortex, and a discrete gliotic...

journal_title：Neuropathology and applied neurobiology

authors： Hunter R,Smith J,Thomson T,Dayan AD

更新日期：1978-07-01 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：: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：:Unless treated with effective antiretroviral therapy many AIDS patients develop a characteristic vacuolar myelopathy of the spinal cord associated with moderate clinical disability. Opinion is divided as to whether vacuolar myelopathy is causally linked to HIV myelitis. To investigate this further, spinal cord patholo...

journal_title：Neuropathology and applied neurobiology

authors： Shepherd EJ,Brettle RP,Liberski PP,Aguzzi A,Ironside JW,Simmonds P,Bell JE

更新日期：1999-02-01 00:00:00

abstract：:In a previous study, we described the inhibitory effects of the growth factor-antagonist, trapidil, on spontaneously occurring oligodendrocyte remyelination in the rat spinal cord following lysolecithin-induced demyelination [30]. The objective of the present study was to further investigate the mechanisms of trapidil...

journal_title：Neuropathology and applied neurobiology

authors： McKay JS,Blakemore WF,Franklin RJ

更新日期：1998-12-01 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：:The organization of astrocytes in myelinated culture of mouse spinal cord tissue was analysed ultrastructurally and compared with the pattern in vivo. Astrocyte cell bodies and their processes, connected by punctate adhesions and gap junctions, formed a continuous layer around the entire explant. Throughout the parenc...

journal_title：Neuropathology and applied neurobiology

authors： Kusaka H,Hirano A,Bornstein MB,Raine CS

更新日期：1984-11-01 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：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：:Paediatric diffuse high-grade gliomas (pHGG) are rare, but deadly tumours. The discovery of recurrent mutations in the tail of histone H3, changing lysine 27 to methionine, or glycine 34 to arginine or valine, has illuminated a critical role for epigenetic dysregulation in the aetiology of childhood gliomas and opened...

journal_title：Neuropathology and applied neurobiology

authors： Kasper LH,Baker SJ

更新日期：2020-02-01 00:00:00

abstract：:A few nanograms of tetanus toxin injected into a rat hippocampus causes a chronic epileptic syndrome characterized by brief seizures that recur intermittently for about 6 weeks. Cognitive and other behavioural impairments persist after the seizures and other epileptic electrographic activity have remitted, and may be ...

journal_title：Neuropathology and applied neurobiology

authors： Jefferys JG,Evans BJ,Hughes SA,Williams SF

更新日期：1992-02-01 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：AIMS:HSPB8 is a small heat shock protein that forms a complex with the co-chaperone BAG3. Overexpression of the HSPB8-BAG3 complex in cells stimulates autophagy and facilitates the clearance of mutated aggregation-prone proteins, whose accumulation is a hallmark of many neurodegenerative disorders. HSPB8-BAG3 could thu...

journal_title：Neuropathology and applied neurobiology

authors： Seidel K,Vinet J,Dunnen WF,Brunt ER,Meister M,Boncoraglio A,Zijlstra MP,Boddeke HW,Rüb U,Kampinga HH,Carra S

更新日期：2012-02-01 00:00:00

abstract：:During an electron microscopic study of the white matter in multiple sclerosis (MS), spheroidal reticular particles were found both in MS and in control brains. These particles have previously been described in the brain in MS and in brain-derived cell cultures in subacute sclerosing panencephalitis. In both cases the...

journal_title：Neuropathology and applied neurobiology

authors： Kirk J,Hutchinson WM

更新日期：1978-09-01 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：:Pathologists have been establishing archives of human organs and tissue for research use for many years now. Controversy has arisen recently over these collections, particularly with regard to the right of patients or relatives to consent to removal and retention of tissue, genetic research using stored tissue samples...

journal_title：Neuropathology and applied neurobiology

authors： Ashcroft R

更新日期：2000-10-01 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：: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：:The effects of exposure to a cytotoxic agent, methylazoxymethanol acetate (MAM Ac), on the distribution, density and quantitative morphology of microglia in the rat forebrain have been examined with the aid of a peroxidase-conjugated lectin derived from Griffonia simplicifolia. Following exposure to MAM Ac (25 mg/kg m...

journal_title：Neuropathology and applied neurobiology

authors： Ashwell KW

更新日期：1992-12-01 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：: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：: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：:Telomerase is a ribonucleoprotein enzyme complex that synthesizes telomere DNA. It is detected in 85-90% of malignant tumours in humans, but not in most somatic cells. Because telomerase plays a critical role in cell immortality, it represents an important target for anticancer therapies. We have previously shown that...

journal_title：Neuropathology and applied neurobiology

authors： Long S,Argyle DJ,Nixon C,Nicholson I,Botteron C,Olby N,Platt S,Smith K,Rutteman GR,Grinwis GC,Nasir L

更新日期：2006-12-01 00:00:00

abstract：UNLABELLED:It is well known that glial cell line-derived neurotrophic factor (GDNF) is a potent neurotrophic factor for motoneurons. We have previously shown that it greatly enhanced motoneuron survival and axon regeneration after implantation of peripheral nerve graft following spinal root avulsion. AIMS:In the curre...

journal_title：Neuropathology and applied neurobiology

authors： Chu TH,Wang L,Guo A,Chan VW,Wong CW,Wu W

更新日期：2012-12-01 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：:There is a great deal of evidence suggesting an important role for systemic inflammation in the pathogenesis of Alzheimer's disease. The role of systemic inflammation, and indeed inflammation in general, is still largely considered to be as a contributor to the disease process rather than of aetiological importance al...

journal_title：Neuropathology and applied neurobiology

authors： Holmes C

更新日期：2013-02-01 00:00:00

abstract：:In this study we describe the effects of trapidil, a putative platelet-derived growth factor-antagonist, on spontaneously occurring remyelination in rat spinal cord. Demyelination was created in the dorsal funiculus of 6- and 11-week-old female rats by the direct injection of 1.0 microliter of 1% lysolecithin. The ani...

journal_title：Neuropathology and applied neurobiology

authors： McKay JS,Blakemore WF,Franklin RJ

更新日期：1997-02-01 00:00:00