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Neuropathology and Applied Neurobiology

Nishant N Vaikath, Daniel Erskine, Christopher M Morris, Nour K Majbour, Kostas Vekrellis, Jia-Yi Li, Omar 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
Henrik Zetterberg, John C van Swieten, Adam L Boxer, Jonathan 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 neuron disease and the atypical parkinsonian disorders, corticobasal syndrome and progressive supranuclear palsy...
November 13, 2018: Neuropathology and Applied Neurobiology
Makis Tzioras, Caitlin Davies, Anastasia Newman, Rosemary Jackson, Tara 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 characterised 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
Sergi Borrego-Écija, Elena Cortés-Vicente, Laura Cervera-Carles, Jordi Clarimón, Josep Gámez, Jordi Batlle, Gerda Ricken, Laura Molina-Porcel, Iban Aldecoa, Raquel Sánchez-Valle, Ricardo Rojas-García, Ellen Gelpi
Abnormal cytoplasmic accumulation of Fused in Sarcoma (FUS) protein is the pathological hallmark of some cases of amyotrophic lateral sclerosis (ALS) with Transactive Response DNA-binding Protein of 43KDa (TDP-43) negative pathology that lack SOD1 mutations. FUS is an RNA-binding protein located predominantly in the nucleus and is involved in regulation of transcription, alternative splicing, RNA stability, microRNA biogenesis, apoptosis and cell division. FUS, Ewing's sarcoma (EWS) and TATA-Binding protein-associated factor 15 (TAF15) proteins constitute the FET (FUS/EWS/TAF15) family, highly conserved and ubiquitously expressed RNA-binding proteins that shuttle between nucleus and cytoplasm assisted by the nuclear import protein Transportin 1 (Trn1)[1]...
October 29, 2018: Neuropathology and Applied Neurobiology
Manuela Neumann, Ian R A Mackenzie
Frontotemporal dementia (FTD) is a heterogeneous clinical syndrome associated with frontotemporal lobar degeneration (FTLD) as a relatively consistent neuropathological hallmark feature. However, the discoveries in the past decade of many of the relevant pathological proteins aggregating in human FTD brains in addition to several new FTD causing gene mutations underlined that FTD is a diverse condition on the neuropathological and genetic basis. This resulted in a novel molecular classification of these conditions based on the predominant protein abnormality and allows most cases of FTD to be placed now into one of three broad molecular subgroups; FTLD with tau, TDP-43 or FET protein accumulation (FTLD-tau, FTLD-TDP and FTLD-FET, respectively)...
October 25, 2018: Neuropathology and Applied Neurobiology
Rianne P Gorter, Jodie Stephenson, Erik Nutma, Jasper Anink, Jenny C de Jonge, Wia Baron, Marie-Christina Jahreiβ, Jeroen A M Belien, Johannes M van Noort, Caroline Mijnsbergen, Eleonora Aronica, Sandra Amor
AIMS: Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterised by progressive loss of motor neurons, muscle weakness, spasticity, paralysis and death usually within 2-5 years of onset. Neuroinflammation is a hallmark of ALS pathology characterized by activation of glial cells, which respond by upregulating small heat shock proteins (HSPBs), but the exact underlying pathological mechanisms are still largely unknown. Here, we investigated the association between ALS disease duration, lower motor neuron loss, TDP-43 pathology, neuroinflammation and HSPB expression...
October 22, 2018: Neuropathology and Applied Neurobiology
Hanbing Ning, Qi Wu, Dongyang Han, Tingting Yao, Jingyun Wang, Wenquan Lu, Shuai Lv, Qiaoyu Jia, Xin Li
BACKGROUND: The prognostic significance of misfolded α-synuclein aggregates in Parkinson's disease has not been well investigated. The aim of this study was to reveal the relationship between misfolded α-synuclein aggregate concentration in cerebrospinal fluid and cognitive decline risk in Parkinson's disease. METHODS: A total of 278 patients with Parkinson's disease were retrospectively included. They were diagnosed between 2011 and 2013. The end point was 2016, and the follow-up period was 54...
October 22, 2018: Neuropathology and Applied Neurobiology
Andrey Korshunov, Belen Casalini, Lukas Chavez, Thomas Hielscher, Martin Sill, Marina Ryzhova, Tanvi Sharma, Daniel Schrimpf, Damian Stichel, David Capper, David E Reuss, Dominik Sturm, Oxana Absalyamova, Andrey Golanov, Sander Lambo, Melanie Bewerunge-Hudler, Peter Lichter, Christel Herold-Mende, Wolfgang Wick, Stefan M Pfister, Marcel Kool, David T W Jones, Andreas von Deimling, Felix Sahm
AIMS: Mutations of isocitrate dehydrogenase (IDH)1/2 affect almost all astrocytomas of WHO grade II and III. A subset of IDH-mutant astrocytic tumours progresses to IDH-mutant glioblastoma or presents with the histology of a glioblastoma at first presentation. We set out here to assess the molecular spectrum of IDH-mutant glioblastomas. METHODS: We performed an integrated molecular analysis of a mono-centric cohort (n = 97); assessed through genome-wide DNA methylation analysis, copy-number profiling, and targeted next generation sequencing using a neurooncology-tailored gene panel...
October 16, 2018: Neuropathology and Applied Neurobiology
Ingmar Blümcke, Roland Coras, Annika K Wefers, David Capper, Eleonora Aronica, Albert Becker, Mrinalini Honavar, Thomas J Stone, Thomas S Jacques, Hajime Miyata, Angelika Mühlebner, Jose Pimentel, Figen Söylemezoğlu, Maria Thom
Low-grade epilepsy-associated brain tumours (LEAT) are the second most common cause for drug-resistant, focal epilepsy, i.e. ganglioglioma (GG) and dysembryoplastic neuroepithelial tumours (DNT). However, molecular pathogenesis, risk factors for malignant progression, and their frequent association with drug-resistant focal seizures remain poorly understood. This contrasts recent progress in understanding the molecular-genetic basis and targeted treatment options in diffuse gliomas. The Neuropathology Task Force of the International League against Epilepsy examined available literature to identify common obstacles in diagnosis and research of LEAT...
October 16, 2018: Neuropathology and Applied Neurobiology
Sebastian Brandner, Zane Jaunmuktane
The discovery of IDH mutations in a subset of glioblastomas 10 years ago has fundamentally changed the approach of brain tumour diagnostics. Soon after this discovery, it emerged that low- and high-grade oligodendrogliomas, diffuse and anaplastic astrocytomas, a proportion of glioblastomas and the now defunct oligoastrocytomas carry mutations in the IDH1 or IDH2 genes. Subsequently, the IDH-mutant tumours were stratified with additional biomarkers, which has led to the definition of the WHO (2016) classes IDH-mutant, 1p/19q codeleted (and TERT promoter mutant) oligodendroglioma and IDH-mutant astrocytoma, with its characteristic loss of ATRX protein expression, assessed by immunostaining ...
October 16, 2018: Neuropathology and Applied Neurobiology
Jack S Bell, Jonathan I Spencer, Richard L Yates, Sydney A Yee, Benjamin M Jacobs, Gabriele C DeLuca
Inflammation and neurodegeneration are key features of many chronic neurological diseases, yet the causative mechanisms underlying these processes are poorly understood. There has been mounting interest in the role of the human microbiome in modulating the inflammatory milieu of the central nervous system in health and disease. To date, most research has focussed on a gut-brain axis, with other mucosal surfaces being relatively neglected. We herein take the novel approach of comprehensively reviewing the roles of the microbiome across several key mucosal interfaces - the nose, mouth, lung, and gut - in health and in Parkinson's disease (PD), Alzheimer's disease (AD) and multiple sclerosis (MS)...
October 8, 2018: Neuropathology and Applied Neurobiology
Akinori Uruha, Yves Allenbach, Jean-Luc Charuel, Lucile Musset, Audrey Aussy, Olivier Boyer, Kuberaka Mariampillai, Océane Landon-Cardinal, Camille Rasmussen, Loïs Bolko, Thierry Maisonobe, Sarah Leonard-Louis, Shigeaki Suzuki, Ichizo Nishino, Werner Stenzel, Olivier Benveniste
AIMS: To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analyzing different DM subforms, and to test the superiority of MxA to other markers. METHODS: Immunohistochemistry for MxA and retinoic acid-inducible gene I (RIG-I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti-Mi-2 (n=6), -TIF1-γ (n=10), -NXP2 (n=13), -MDA5 (n=10), or -SAE (n=1) autoantibodies and with no detectable autoantibody (n=17)...
September 28, 2018: Neuropathology and Applied Neurobiology
L Mahady, M Nadeem, M Malek-Ahmadi, K Chen, S E Perez, E J Mufson
AIMS: Alzheimer's disease (AD) is characterized by degeneration of cholinergic basal forebrain (CBF) neurons in the nucleus basalis of Meynert (nbM), which provides the major cholinergic input to the cortical mantle and is related to cognitive decline in patients with AD. Cortical histone deacetylase (HDAC) dysregulation has been associated with neuronal degeneration during AD progression. However, whether HDAC alterations play a role in CBF degeneration during AD onset is unknown. We investigated global HDAC protein levels and nuclear HDAC2 immunoreactivity in tissue containing the nbM, changes and their association with neurofibrillary tangles (NFTs) during the progression of AD...
September 25, 2018: Neuropathology and Applied Neurobiology
R Guerreiro, T Orme, A C Naj, A B Kuzma, G D Schellenberg, J Bras
No abstract text is available yet for this article.
September 19, 2018: Neuropathology and Applied Neurobiology
L Winter, A Unger, C Berwanger, M Spörrer, M Türk, F Chevessier, K-H Strucksberg, U Schlötzer-Schrehardt, I Wittig, W H Goldmann, K Marcus, W A Linke, C S Clemen, R Schröder
AIMS: We investigated newly generated immortalized heterozygous and homozygous R349P desmin knock-in myoblasts in conjunction with the corresponding desminopathy mice as models for desminopathies to analyse major protein quality control processes in response to the presence of R349P mutant desmin. METHODS: We used hetero- and homozygous R349P desmin knock-in mice for analyses and for crossbreeding with p53 knock-out mice to generate immortalized R349P desmin knock-in skeletal muscle myoblasts and myotubes...
September 4, 2018: Neuropathology and Applied Neurobiology
J A Cotter, A R Judkins
No abstract text is available yet for this article.
October 2018: Neuropathology and Applied Neurobiology
S Lehmann, E Esch, P Hartmann, A Goswami, S Nikolin, J Weis, C Beyer, S Johann
AIMS: Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motoneurons and progressive muscle wasting. Inflammatory processes, mediated by non-neuronal cells, such as glial cells, are known to contribute to disease progression. Inflammasomes consist of pattern recognition receptors (PRRs), apoptosis-associated speck-like protein (ASC) and caspase 1 and are essential for interleukin (IL) processing and a rapid immune response after tissue damage. Recently, we described inflammasome activation in the spinal cord of ALS patients and in SOD1(G93A) ALS mice...
October 2018: Neuropathology and Applied Neurobiology
A Hoshi, A Tsunoda, T Yamamoto, M Tada, A Kakita, Y Ugawa
AIMS: Glutamate neurotoxicity plays an important role in the pathogenesis of various neurodegenerative disorders. Many studies have demonstrated that glutamate transporter-1 (GLT-1), the dominant astrocytic glutamate transporter, is significantly reduced in the cerebral cortex of patients with Alzheimer's disease (AD), suggesting that glutamate-mediated excitotoxicity might contribute to the pathogenesis of AD. In a previous study, we have demonstrated marked alterations in the expression of the astrocytic water channel protein aquaporin-4 (AQP4) in relation to amyloid β deposition in human AD brains...
October 2018: Neuropathology and Applied Neurobiology
A Franceschini, R Strammiello, S Capellari, A Giese, P Parchi
AIMS: The aim of this study was to describe the regional profiles of microglial activation in sporadic Creutzfeldt-Jakob disease (sCJD) subtypes and analyse the influence of prion strain, disease duration and codon 129 genotype. METHODS: We studied the amount/severity and distribution of activated microglia, protease-resistant prion protein (PrPSc ) spongiform change, and astrogliosis in eight regions of 57 brains, representative of the entire spectrum of sCJD subtypes...
October 2018: Neuropathology and Applied Neurobiology
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