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    1828 results match your criteria Brain Pathology[Journal]

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    Fetal microchimerism in human brain tumors.
    Brain Pathol 2017 Sep 18. Epub 2017 Sep 18.
    Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO.
    Sex differences in cancer incidence and survival, including central nervous system tumors, are well documented. Multiple mechanisms contribute to sex differences in health and disease. Recently, the presence of fetal-in-maternal microchimeric cells has been shown to have prognostic significance in breast and colorectal cancers. Read More

    HIPPOCAMPAL SCLEROSIS, HIPPOCAMPAL NEURON LOSS PATTERNS AND TDP-43 IN THE AGED POPULATION.
    Brain Pathol 2017 Aug 18. Epub 2017 Aug 18.
    Institute of Public Health, University of Cambridge, Cambridge, UK.
    Hippocampal neuron loss is a common neuropathological feature in old age with various underlying aetiologies. Hippocampal sclerosis of aging (HS-Aging) is neuropathologically characterized by severe CA1 neuronal loss and frequent presence of transactive response DNA-binding protein of 43kDa (TDP-43) aggregations. Its aetiology is unclear and currently no standardized approaches to measure HS-Aging exist. Read More

    Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?
    Brain Pathol 2017 Aug 19. Epub 2017 Aug 19.
    Department of (Neuro)Pathology, Academic Medical Center, Amsterdam, The Netherlands.
    Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. Read More

    Protein astrogliopathies in human neurodegenerative diseases and aging.
    Brain Pathol 2017 Sep;27(5):675-690
    Center for Neurodegenerative Disease Research, Institute on Aging and Department of Pathology and Laboratory Medicine of the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
    Neurodegenerative diseases are characterized by progressive dysfunction and loss of neurons associated with depositions of pathologically altered proteins showing hierarchical involvement of brain regions. The role of astrocytes in the pathogenesis of neurodegenerative diseases is explored as contributors to neuronal degeneration or neuroprotection pathways, and also as potential mediators of the transcellular spreading of disease-associated proteins. Protein astrogliopathy (PAG), including deposition of amyloid-β, prion protein, tau, α-synuclein, and very rarely transactive response DNA-binding protein 43 (TDP-43) is not unprecedented or unusual in neurodegenerative diseases. Read More

    Stratification of astrocytes in healthy and diseased brain.
    Brain Pathol 2017 Sep;27(5):629-644
    Department of Neurobiology, Civitan International Research Center and Center for Glial Biology in Medicine, Evelyn F. McKnight Brain Institute, Atomic Force Microscopy & Nanotechnology Laboratories, 1719 6th Avenue South, CIRC 429, University of Alabama at Birmingham, Birmingham, AL 35294-0021.
    Astrocytes, a subtype of glial cells, come in variety of forms and functions. However, overarching role of these cell is in the homeostasis of the brain, be that regulation of ions, neurotransmitters, metabolism or neuronal synaptic networks. Loss of homeostasis represents the underlying cause of all brain disorders. Read More

    Diversity of astroglial responses across human neurodegenerative disorders and brain aging.
    Brain Pathol 2017 Sep;27(5):645-674
    Department of Pathology and Experimental Therapeutics, University of Barcelona, Barcelona, Spain.
    Astrogliopathy refers to alterations of astrocytes occurring in diseases of the nervous system, and it implies the involvement of astrocytes as key elements in the pathogenesis and pathology of diseases and injuries of the central nervous system. Reactive astrocytosis refers to the response of astrocytes to different insults to the nervous system, whereas astrocytopathy indicates hypertrophy, atrophy/degeneration and loss of function and pathological remodeling occurring as a primary cause of a disease or as a factor contributing to the development and progression of a particular disease. Reactive astrocytosis secondary to neuron loss and astrocytopathy due to intrinsic alterations of astrocytes occur in neurodegenerative diseases, overlap each other, and, together with astrocyte senescence, contribute to disease-specific astrogliopathy in aging and neurodegenerative diseases with abnormal protein aggregates in old age. Read More

    Alterations in the steroid biosynthetic pathways in the human prefrontal cortex in mood disorders: A post-mortem study.
    Brain Pathol 2017 Jul 27. Epub 2017 Jul 27.
    Netherlands Institute for Neuroscience, An Institute of the Royal Netherlands Academy of Arts and Sciences, Amsterdam, the Netherlands.
    Altered levels of steroids have been reported in the brain, cerebral spinal fluid and plasma of patients with mood disorders. Neuroimaging studies have reported both functional and structural alterations in mood disorders, for instance in the anterior cingulate cortex (ACC) and dorsolateral prefrontal cortex (DLPFC). In order to determine whether the endogenous production of steroids is altered in the ACC and DLPFC of patients with major depressive disorder (MDD) or bipolar disorder (BPD), quantitative real-time PCR was performed to detect mRNA expression level of key enzymes in the steroid biosynthetic pathways. Read More

    Tissue microarray methodology identifies complement pathway activation and dysregulation in progressive multiple sclerosis.
    Brain Pathol 2017 Jul 14. Epub 2017 Jul 14.
    Division of Infection and Immunity, Henry Wellcome Building, Cardiff University, Cardiff, CF14 4XW, United Kingdom.
    The complement pathway has potential contributions to both white (WM) and grey matter (GM) pathology in Multiple Sclerosis (MS). A quantitative assessment of complement involvement is lacking. Here we describe the use of Tissue MicroArray (TMA) methodology in conjunction with immunohistochemistry to investigate the localization of complement pathway proteins in progressive MS cortical GM and subcortical WM. Read More

    Autophagy in neurodegenerative diseases: pathogenesis and therapy.
    Brain Pathol 2017 Jul 13. Epub 2017 Jul 13.
    The Key Laboratory of Stem Cell Biology and Neurogenomic Laboratory, Institute of Health Sciences, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences & Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.
    The most prevalent pathological features of many neurodegenerative diseases are the aggregation of misfolded proteins and the loss of certain neuronal populations. Autophagy, as major intracellular machinery for degrading aggregated proteins and damaged organelles, has been reported to be involved in the occurrence of pathological changes in many neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, Huntington's disease and amyotrophic lateral sclerosis. In this review, we summarize most recent research progress in this topic and provide a new perspective regarding autophagy regulation on the pathogenesis of neurodegenerative diseases. Read More

    Understanding neurodevelopmental disorders using human pluripotent stem cell-derived neurons.
    Brain Pathol 2017 Jul;27(4):508-517
    Neuroscience and Mental Health Research Institute, School of Medicine and School of Biosciences, Cardiff University, Cardiff, United Kingdom.
    Research into psychiatric disorders has long been hindered by the lack of appropriate models. Induced pluripotent stem cells (iPSCs) offer an unlimited source of patient-specific cells, which in principle can be differentiated into all disease-relevant somatic cell types to create in vitro models of the disorder of interest. Here, neuronal differentiation protocols available for this purpose and the current progress on iPSCs-based models of schizophrenia, autism spectrum disorders and bipolar disorder were reviewed. Read More

    Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.
    Brain Pathol 2017 Jul;27(4):518-524
    MRC Centre for Regenerative Medicine, University of Edinburgh, Edinburgh, EH16 4UU, UK.
    C9ORF72 repeat expansion is the most frequent causal genetic mutation giving rise to amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD). The relatively recent discovery of the C9ORF72 repeat expansion in 2011 and the complexity of the mutation have meant that animal models that successfully recapitulate human C9ORF72 repeat expansion-mediated disease are only now emerging. Concurrent advances in the use of patient-derived induced pluripotent stem cells (iPSCs) to model aspects of neurological disease offers an additional approach for the study of C9ORF72 mutation. Read More

    Modeling tau pathology in human stem cell derived neurons.
    Brain Pathol 2017 Jul;27(4):525-529
    Department of Molecular Neuroscience, UCL Institute of Neurology, London, WC1N 1PJ, UK.
    Tau pathology is a defining characteristic of multiple neurodegenerative disorders including Alzheimer's disease (AD) and Frontotemporal Dementia (FTD) with tau pathology. There is strong evidence from genetics and experimental models to support a central role for tau dysfunction in neuronal death, suggesting tau is a promising therapeutic target for AD and FTD. However, the development of tau pathology can precede symptom onset by several years, so understanding the earliest molecular events in tauopathy is a priority area of research. Read More

    Modeling Parkinson's disease with induced pluripotent stem cells harboring α-synuclein mutations.
    Brain Pathol 2017 Jul;27(4):545-551
    MRC Centre for Regenerative Medicine, Institute for Stem Cell Research, School of Biological Sciences, The University of Edinburgh, Edinburgh, EH16 4UU, UK.
    Parkinson's disease (PD) is a common neurodegenerative condition affecting more than 8 million people worldwide. Although, the majority of PD cases are sporadic in nature, there are a growing number of monogenic mutations identified to cause PD in a highly penetrant manner. Many of these familial mutations give rise to a condition that is clinically and neuropathologically similar, if not identical, to sporadic PD. Read More

    Astrocytes in a dish: Using pluripotent stem cells to model neurodegenerative and neurodevelopmental disorders.
    Brain Pathol 2017 Jul;27(4):530-544
    Trinity College Institute for Neuroscience, Trinity College Dublin 2, Ireland.
    Neuroscience and Neurobiology have historically been neuron biased, yet up to 40% of the cells in the brain are astrocytes. These cells are heterogeneous and regionally diverse but universally essential for brain homeostasis. Astrocytes regulate synaptic transmission as part of the tripartite synapse, provide metabolic and neurotrophic support, recycle neurotransmitters, modulate blood flow and brain blood barrier permeability and are implicated in the mechanisms of neurodegeneration. Read More

    TGFβ pathway deregulation and abnormal phospho-SMAD2/3 staining in hereditary cerebral hemorrhage with amyloidosis-Dutch type.
    Brain Pathol 2017 May 29. Epub 2017 May 29.
    Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands.
    Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) is an early onset hereditary form of cerebral amyloid angiopathy (CAA) pathology, caused by the E22Q mutation in the amyloid β (Aβ) peptide. Transforming growth factor β1 (TGFβ1) is a key player in vascular fibrosis and in the formation of angiopathic vessels in transgenic mice. Therefore, we investigated whether the TGFβ pathway is involved in HCHWA-D pathogenesis in human postmortem brain tissue from frontal and occipital lobes. Read More

    Rapid amyloid-β oligomer and protofibril accumulation in traumatic brain injury.
    Brain Pathol 2017 May 29. Epub 2017 May 29.
    Department of Neuroscience, Section of Neurosurgery, Uppsala University, Uppsala, Sweden.
    Deposition of amyloid-β (Aβ) is central to Alzheimer's disease (AD) pathogenesis and associated with progressive neurodegeneration in traumatic brain injury (TBI). We analyzed predisposing factors for Aβ deposition including monomeric Aβ40, Aβ42 and Aβ oligomers/protofibrils, Aβ species with pronounced neurotoxic properties, following human TBI. Highly selective ELISAs were used to analyze N-terminally intact and truncated Aβ40 and Aβ42, as well as Aβ oligomers/protofibrils, in human brain tissue, surgically resected from severe TBI patients (n = 12; mean age 49. Read More

    Preclinical transgenic and patient-derived xenograft models recapitulate the radiological features of human adamantinomatous craniopharyngioma.
    Brain Pathol 2017 May 8. Epub 2017 May 8.
    Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, UK.
    To assess the clinical relevance of transgenic and patient-derived xenograft models of adamantinomatous craniopharyngioma (ACP) using serial magnetic resonance imaging (MRI) and high resolution post-mortem microcomputed tomography (μ-CT), with correlation with histology and human ACP imaging. The growth patterns and radiological features of tumors arising in Hesx1(Cre/+) ;Ctnnb1(lox(ex3)/+) transgenic mice, and of patient-derived ACP xenografts implanted in the cerebral cortex, were monitored longitudinally in vivo with anatomical and functional MRI, and by ex vivo μ-CT at study end. Pathological correlates with hematoxylin and eosin stained sections were investigated. Read More

    Loss of SMARCE1 expression is a specific diagnostic marker of clear cell meningioma: a comprehensive immunophenotypical and molecular analysis.
    Brain Pathol 2017 May 5. Epub 2017 May 5.
    Department of Neuropathology, Sainte-Anne Hospital, 75014, Paris, France.
    Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. Read More

    Pericyte-derived bone morphogenetic protein 4 underlies white matter damage after chronic hypoperfusion.
    Brain Pathol 2017 May 4. Epub 2017 May 4.
    Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
    Subcortical small vessel disease (SVD) is characterized by white matter damage resulting from arteriolosclerosis and chronic hypoperfusion. Transforming growth factor beta 1 (TGFB1) is dysregulated in the hereditary SVD, CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy). However, very little is known about the role of the largest group in the TGFB superfamily - the bone morphogenetic proteins (BMPs) - in SVD pathogenesis. Read More

    Cytotoxic CD8(+) T cell ablation enhances the capacity of regulatory T cells to delay viral elimination in Theiler's murine encephalomyelitis.
    Brain Pathol 2017 Apr 27. Epub 2017 Apr 27.
    Department of Pathology, University of Veterinary Medicine Hannover, Hannover, Germany.
    Theiler's murine encephalomyelitis (TME) of susceptible mouse strains is a commonly used infectious animal model for multiple sclerosis. The study aim was to test the hypothesis whether cytotoxic T cell responses account for the limited impact of regulatory T cells on antiviral immunity in TME virus-induced demyelinating disease (TMEV-IDD) resistant C57BL/6 mice. TME virus-infected C57BL/6 mice were treated with (i) interleukin-2/-anti-interleukin-2-antibody-complexes to expand regulatory T cells ("Treg-expansion"), (ii) anti-CD8-antibodies to deplete cytotoxic T cells ("CD8-depletion") or (iii) with a combination of Treg-expansion and CD8-depletion ("combined treatment") prior to infection. Read More

    Genetically engineered mouse models of craniopharyngioma: an opportunity for therapy development and understanding of tumor biology.
    Brain Pathol 2017 May;27(3):364-369
    Developmental Biology and Cancer, UCL Great Ormond Street Institute of Child Health, University College London, Guilford Street, London, WC1N 1EH, UK.
    Adamantinomatous craniopharyngioma (ACP) is the commonest tumor of the sellar region in childhood. Two genetically engineered mouse models have been developed and are giving valuable insights into ACP biology. These models have identified novel pathways activated in tumors, revealed an important function of paracrine signalling and extended conventional theories about the role of organ-specific stem cells in tumorigenesis. Read More

    Proteomics in pediatric cystic craniopharyngioma.
    Brain Pathol 2017 May;27(3):370-376
    Istituto di Chimica del Riconoscimento Molecolare, Consiglio Nazionale delle Ricerche, Rome, Italy.
    Adamantinomatous craniopharyngioma (ACP) is still often burdened by a poor prognosis in children as far as the risk of recurrence and the quality of life are concerned. Therefore, many efforts are now dedicated to investigate the molecular characteristics of this tumor aiming at finding new therapeutic options. ACP is prevalently a cystic lesion so that an increasing number of researches are focused on the analysis of its cystic content. Read More

    Models of human adamantinomatous craniopharyngioma tissue: Steps toward an effective adjuvant treatment.
    Brain Pathol 2017 May;27(3):358-363
    Institute of Pathology, Sozialstiftung Bamberg, Buger Str. 80, Bamberg, 96049, Germany.
    Even though ACP is a benign tumor, treatment is challenging because of the tumor's eloquent location. Today, with the exception of surgical intervention and irradiation, further treatment options are limited. However, ongoing molecular research in this field provides insights into the pathways involved in ACP pathogenesis and reveal a plethora of druggable targets. Read More

    Axonal loss in the multiple sclerosis spinal cord revisited.
    Brain Pathol 2017 Apr 12. Epub 2017 Apr 12.
    Blizard Institute (Neuroscience), Barts and the London School of Medicine & Dentistry, Queen Mary University of London, London, UK.
    Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area (CSA) assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. Read More

    Characterising subtypes of hippocampal sclerosis and reorganization: correlation with pre and postoperative memory deficit.
    Brain Pathol 2017 Apr 5. Epub 2017 Apr 5.
    Department of Clinical and Experimental Epilepsy, UCL Institute of Neurology, Queen Square, London, WCN1BG, UK.
    Neuropathological subtypes of hippocampal sclerosis (HS) in temporal lobe epilepsy (The 2013 International League Against Epilepsy classification) are based on the qualitative assessment of patterns of neuronal loss with NeuN. In practice, some cases appear indeterminate between type 1 (CA1 and CA4 loss) and type 2 HS (CA1 loss) and we predicted that MAP2 would enable a more stringent classification. HS subtypes, as well as the accompanying alteration of axonal networks, regenerative capacity and neurodegeneration have been previously correlated with outcome and memory deficits and may provide prognostic clinical information. Read More

    Transcriptional network analysis in frontal cortex in Lewy body diseases with focus on dementia with Lewy bodies.
    Brain Pathol 2017 Mar 21. Epub 2017 Mar 21.
    Department of Pathology and Experimental Therapeutics, University of Barcelona, L'Hospitalet de Llobregat, Spain.
    The present study investigates global transcriptional changes in frontal cortex area 8 in incidental Lewy Body disease (iLBD), Parkinson disease (PD) and Dementia with Lewy bodies (DLB). We identified different coexpressed gene sets associated with disease stages, and gene ontology categories enriched in gene modules and differentially expressed genes including modules or gene clusters correlated to iLBD comprising upregulated dynein genes and taste receptors, and downregulated innate inflammation. Focusing on DLB, we found modules with genes significantly enriched in functions related to RNA and DNA production, mitochondria and energy metabolism, purine metabolism, chaperone and protein folding system and synapses and neurotransmission (particularly the GABAergic system). Read More

    Intracranial myxoid mesenchymal tumors with EWSR1-CREB family gene fusions: myxoid variant of angiomatoid fibrous histiocytoma or novel entity?
    Brain Pathol 2017 Mar 9. Epub 2017 Mar 9.
    Department of Pathology, Boston Children's Hospital, Boston, MA.
    Intracranial myxoid mesenchymal tumor harboring EWSR1 fusions with CREB family of genes was recently described, and it resembles the myxoid variant of angiomatoid fibrous histiocytoma. We present three pediatric patients with intracranial EWSR1-rearranged myxoid mesenchymal neoplasm and provide a molecular genetic characterization of these tumors. Clinical histories and imaging results were reviewed. Read More

    Overlapping but distinct TDP-43 and tau pathologic patterns in aged hippocampi.
    Brain Pathol 2017 Mar 9. Epub 2017 Mar 9.
    Department of Pathology and Laboratory Medicine, University of Kentucky, Lexington, KY.
    Intracellular proteinaceous aggregates (inclusion bodies) are almost always detectable at autopsy in brains of elderly individuals. Inclusion bodies composed of TDP-43 and tau proteins often coexist in the same brain, and each of these pathologic biomarkers is associated independently with cognitive impairment. However, uncertainties remain about how the presence and neuroanatomical distribution of inclusion bodies correlate with underlying diseases including Alzheimer's disease (AD). Read More

    Cerebellar compartmentation of prion pathogenesis.
    Brain Pathol 2017 Mar 7. Epub 2017 Mar 7.
    Cytologie et Cytopathologie Neuronales, Institut des Neurosciences Cellulaires & Intégratives, CNRS UPR 3212, Strasbourg, France.
    In prion diseases, the brain lesion profile is influenced by the prion "strain" properties, the invasion route to the brain, and still unknown host cell-specific parameters. To gain insight into those endogenous factors, we analyzed the histopathological alterations induced by distinct prion strains in the mouse cerebellum. We show that 22L and ME7 scrapie prion proteins (PrP(22L) , PrP(ME7) ), but not bovine spongiform encephalopathy PrP(6PB1) , accumulate in a reproducible parasagittal banding pattern in the cerebellar cortex of infected mice. Read More

    Review of xanthomatous lesions of the sella.
    Brain Pathol 2017 May;27(3):377-395
    Department of Neurosurgery, University of Colorado Anschutz Medical Campus, Aurora, CO.
    Xanthomatous lesions of the sellar region have traditionally been divided into two separate categories, xanthomatous hypophysitis (XH) and xanthogranuloma (XG) of the sellar region. The seminal article on XH, a condition typified by foamy histiocytes and lymphoplasmacytic infiltrates in the pituitary gland/sellar region, but usually little or no hemosiderin pigment, detailed three patients. However, most reports since that time have been single cases, making understanding of the entity difficult. Read More


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