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Neuropathology and Applied Neurobiology

S B Wharton, N S Verber, B E Wagner, J R Highley, D J Fillingham, R Waller, K Strand, P G Ince, P J Shaw
AIMS: Amyotrophic lateral sclerosis / motor neurone disease (ALS/MND) is characterised by the presence of inclusions containing TDP-43 within motor neurones. In rare cases, ALS/MND may be associated with inclusions containing other proteins, such as fused in sarcoma (FUS), whilst motor system pathology may rarely be a feature of other neurodegenerative disorders. We here have investigated the association of FUS and tau pathology. METHODS: We report a case with an ALS/MND-plus clinical syndrome which pathologically demonstrated both FUS pathology and an atypical tauopathy...
January 19, 2019: Neuropathology and Applied Neurobiology
Raquel Cuevas-Diaz Duran, Haichao Wei, Dong H Kim, Jia Qian Wu
Genome-wide transcriptional studies have demonstrated that tens of thousands of lncRNA genes are expressed in the CNS and that they exhibit tissue- and cell-type specificity. Their regulated and dynamic expression, and their co-expression with protein-coding gene neighbours, have led to the study of the functions of lncRNAs in CNS development and disorders. In this review, we describe the general characteristics, localization, and classification of lncRNAs. We also elucidate examples of the molecular mechanisms of nuclear and cytoplasmic lncRNA actions in the CNS and discuss common experimental approaches used to identify and unveil the functions of lncRNAs...
January 13, 2019: Neuropathology and Applied Neurobiology
Cheryl Reeves, Anaclara Pradim Jardim, Sanjay M Sisodiya, Maria Thom, Joan Y W Liu
AIMS: Understanding the spatiotemporal dynamics of reactive cell types following brain injury is important for future therapeutic interventions. We have previously used penetrating cortical injuries following intracranial recordings as a brain repair model to study scar-forming nestin-expressing cells. We now explore the relationship between nestin-expressing cells, PDGFRβ+ pericytes and Olig2+ glia, including their proliferation and functional maturation. METHODS: In 32 cases, ranging from 3 to 461 days post injury (dpi), immunohistochemistry for PDGFRβ, nestin, GFAP, Olig2, MCM2, Aquaporin 4 (Aq4), Glutamine Synthetase (GS), and Connexin 43 (Cx43) were quantified for cell densities, labelling index (LI) and cellular co-expression at the injury site compared to control regions...
January 12, 2019: Neuropathology and Applied Neurobiology
Ana Artero Castro, Candela Machuca Arellano, Francisco Javier Rodriguez Jimenez, Pavla Jendelova, Slaven Erceg
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset neurodegenerative disease that includes progressive cerebellar dysfunction. ARSACS is caused by an autosomal recessive loss-of-function mutation in the SACS gene, which encodes for SACSIN. Although animal models are still necessary to investigate the role of SACSIN in the pathology of this disease, more reliable human cellular models need to be generated to better understand the cerebellar pathophysiology of ARSACS. The discovery of human induced pluripotent stem cells (hiPSC) has permitted the derivation of patient-specific cells...
January 12, 2019: Neuropathology and Applied Neurobiology
Gianluigi Forloni, Roberto Chiesa, Orso Bugiani, Mario Salmona, Fabrizio Tagliavini
A quarter of a century ago, we proposed an innovative approach to studying the pathogenesis of prion disease, one of the most intriguing biomedical problems that remains unresolved. The synthesis of a peptide homologous to residues 106-126 of the human prion protein (PrP106-126), a sequence present in the PrP amyloid protein of Gerstmann-Sträussler-Scheinker syndrome patients, provided a tractable tool for investigating the mechanisms of neurotoxicity. Together with several other discoveries at the beginning of the 1990s, PrP106-126 contributed to underpinning the role of amyloid in the pathogenesis of protein-misfolding neurodegenerative disorders...
January 12, 2019: Neuropathology and Applied Neurobiology
Rhian Convery, Simon Mead, Jonathan D Rohrer
Frontotemporal dementia (FTD) is a heterogeneous group of disorders causing neurodegeneration within a network of areas centred on the frontal and temporal lobes. Clinically, patients present with behavioural symptoms (behavioural variant FTD) or language disturbance (primary progressive aphasia), although there is an overlap with motor neurone disease and atypical parkinsonian disorders. Whilst neuroimaging commonly reveals abnormalities in the frontal and temporal lobes, a closer review identifies a more complex picture with a variable asymmetry of neuronal loss, widespread subcortical involvement, and in many cases more posterior cortical atrophy...
December 24, 2018: Neuropathology and Applied Neurobiology
Daniel A Solomon, Jacqueline Mitchell, Marie-Therese Konrad, Caroline Vance, Sarah Mizielinska
Frontotemporal dementia (FTD) encompasses a collection of clinically and pathologically diverse neurological disorders. Clinical features of behavioural and language dysfunction are associated with neurodegeneration, predominantly of frontal and temporal cortices. Over the past decade there has been significant advances in the understanding of the genetic aetiology and neuropathology of FTD which have led to the creation of various disease models to investigate the molecular pathways that contribute to disease pathogenesis...
December 23, 2018: Neuropathology and Applied Neurobiology
Barbara Borroni, Antonella Alberici, Emanuele Buratti
Frontotemporal Lobar Degeneration (FTLD) is a group of disorders that principally affect the frontal and temporal lobes of the brain. In many parts of the world, FTLD is rapidly becoming a serious health burden on society and, as a result, the molecular mechanisms that underlie its onset and development have been the target of intense research efforts in recent years. Nonetheless, despite crucial pathological and genetic discoveries in this area much is still uncertain about how the many genes associated with this disease cause the observed neurodegeneration...
December 21, 2018: Neuropathology and Applied Neurobiology
Peter S Spencer, Glen Kisby
Leija-Salazar and colleagues discuss current evidence for somatic mutations in neurodegeneration, focusing on sporadic, usually late-onset neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS), Parkinson disease (PD) and Alzheimer's disease (AD). Somatic mutation, defined as any acquired post-zygotic change in the nuclear genome, has long been linked to chemically induced DNA damage, aging and cancer but little more than a theoretical basis exists to associate comparable phenomena with sporadic neurodegenerative diseases...
December 17, 2018: Neuropathology and Applied Neurobiology
Anneliese Krell, Marietta Wolter, Nina Stojcheva, Caroline Hertler, Franziska Liesenberg, Marc Zapatka, Michael Weller, Bastian Malzkorn, Guido Reifenberger
AIMS: Aberrant expression of microRNAs (miRNAs) is frequent in various cancers including gliomas. We aimed to characterize the role of miR-16-5p as a candidate tumour suppressor miRNA in gliomas. METHODS: Real-time PCR-based approaches were used for miRNA and mRNA expression profiling of glioma and non-neoplastic brain tissues as well as glioma cell lines. Protein levels were determined by Western blotting. In vitro analyses were performed following overexpression of miR-16-5p, trichostatin A treatment, and siRNA-mediated knock-down of HDAC3 in glioma cells...
December 13, 2018: Neuropathology and Applied Neurobiology
T Revesz, D M Mann, T Lashley
No abstract text is available yet for this article.
February 2019: Neuropathology and Applied Neurobiology
(no author information available yet)
No abstract text is available yet for this article.
December 2018: Neuropathology and Applied Neurobiology
S Koga, W-L Lin, R L Walton, O A Ross, D W Dickson
AIMS: This study aimed to assess clinicopathologic features of transactive response DNA-binding protein of 43 kDa (TDP-43) pathology and its risk factors in multiple system atrophy (MSA). METHODS: Paraffin-embedded sections of the amygdala and basal forebrain from 186 autopsy-confirmed MSA cases were screened with immunohistochemistry for phospho-TDP-43. In cases having TDP-43 pathology, additional brain regions were assessed. Immunohistochemical and immunofluorescence double-staining and immunogold electron microscopy (IEM) were performed to evaluate colocalization of TDP-43 and α-synuclein...
December 2018: Neuropathology and Applied Neurobiology
S C Shelmerdine, J C Hutchinson, S Al-Sarraj, N Cary, T Dawson, D Du Plessis, P G Ince, S McLaughlin, L Palm, C Smith, N Stoodley, R van Rijn, O J Arthurs, T S Jacques
AIMS: To develop an expert consensus statement regarding appropriate clinical and forensic post mortem neurological imaging. METHODS: An expert panel of clinicians were recruited from registered members of the British Neuropathological Society (BNS) and the International Society of Forensic Radiology and Imaging (ISFRI) with post mortem expertise. Following a focus group meeting, 16 core statements were incorporated into an online modified Delphi survey and each panellist was asked to score their level of agreement...
December 2018: Neuropathology and Applied Neurobiology
J B Iorgulescu, S Ferris, A Agarwal, S Casavilca Zambrano, D A Hill, R Schmidt, A Perry
No abstract text is available yet for this article.
December 2018: Neuropathology and Applied Neurobiology
N N Vaikath, D Erskine, C M Morris, N K Majbour, K Vekrellis, J-Y Li, O M A El-Agnaf
AIMS: Lewy body diseases are neuropathologically characterized by the abnormal accumulation of α-synuclein (α-syn) protein within vulnerable neurons. Although studies have evaluated α-syn in post mortem brain tissue, previous findings have been limited by typically employing pan-α-syn antibodies that may not recognize disease-relevant forms of protein. We investigated the presence of α-syn species present in post mortem brain tissues from Lewy body disease and Alzheimer's disease...
November 13, 2018: Neuropathology and Applied Neurobiology
H Zetterberg, J C van Swieten, A L Boxer, J D Rohrer
Frontotemporal dementias (FTDs) are clinically, genetically and pathologically heterogeneous neurodegenerative disorders that affect the frontal and anterior temporal lobes of the brain. They are relatively common causes of young-onset dementia and usually present with behavioural disturbance (behavioural variant FTD) or language impairment (primary progressive aphasia), but there is also overlap with motor neurone disease and the atypical parkinsonian disorders, corticobasal syndrome and progressive supranuclear palsy...
November 13, 2018: Neuropathology and Applied Neurobiology
M Tzioras, C Davies, A Newman, R Jackson, T Spires-Jones
Despite more than a century of research, the aetiology of sporadic Alzheimer's disease (AD) remains unclear and finding disease modifying treatments for AD presents one of the biggest medical challenges of our time. AD pathology is characterized by deposits of aggregated amyloid beta (Aβ) in amyloid plaques and aggregated tau in neurofibrillary tangles. These aggregates begin in distinct brain regions and spread throughout the brain in stereotypical patterns. Neurodegeneration, comprising loss of synapses and neurons, occurs in brain regions with high tangle pathology, and an inflammatory response of glial cells appears in brain regions with pathological aggregates...
November 5, 2018: Neuropathology and Applied Neurobiology
Shireena A Yasin, Peter W Schutz, Claire T Deakin, Erdal Sag, Hemlata Varsani, Stefanie Simou, Lucy R Marshall, Sarah L Tansley, Neil J McHugh, Janice L Holton, Lucy R Wedderburn, Thomas S Jacques
AIM: Juvenile idiopathic inflammatory myopathies (IIM) have been recently reclassified into clinico-serological subgroups. Myopathological correlates of the subgroups are incompletely understood. METHODS: We studied muscle biopsies from 101 children with clinically and serologically-defined juvenile IIM from the UK JDM Cohort and Biomarker Study by applying the international JDM score tool, myopathological review, and C5b-9 complement analysis. RESULTS: Autoantibody data were available for 90/101 cases with 18/90 cases positive for anti-TIF1γ, 15/90 anti-NXP2, 11/90 anti-MDA5, 5/90 anti-Mi2, and 6/90 anti-PmScl...
October 31, 2018: Neuropathology and Applied Neurobiology
S Borrego-Écija, E Cortés-Vicente, L Cervera-Carles, J Clarimón, J Gámez, J Batlle, G Ricken, L Molina-Porcel, I Aldecoa, R Sánchez-Valle, R Rojas-García, E Gelpi
No abstract text is available yet for this article.
October 29, 2018: Neuropathology and Applied Neurobiology
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