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https://www.readbyqxmd.com/read/28214318/clinical-course-treatment-modalities-and-quality-of-life-in-patients-with-congenital-melanocytic-nevi-data-from-the-german-cmn-registry
#1
Maria Elisabeth Wramp, Anna Langenbruch, Matthias Augustin, Detlef Zillikens, Sven Krengel
BACKGROUND: Congenital melanocytic nevi (CMN) are associated with mental stress as well as medical risks for those affected. The German CMN registry was initiated in 2005. Herein, we present results from an interim analysis focusing on disease course, treatment modalities, and quality of life. PATIENTS AND METHODS: One hundred patients enrolled in the registry between 2005 and 2012 were included in this prospective cohort study, and asked to participate in a follow-up survey...
February 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28214109/a-novel-mutation-in-trem2-gene-causing-nasu-hakola-disease-and-review-of-the-literature
#2
Efthimios Dardiotis, Vasileios Siokas, Eva Pantazi, Maria Dardioti, Dimitrios Rikos, Georgia Xiromerisiou, Aikaterini Markou, Dimitra Papadimitriou, Matthaios Speletas, Georgios M Hadjigeorgiou
Nasu-hakola disease (NHD) is a rare disease characterized by bone cysts and fractures, frontal lobe syndrome, and progressive presenile dementia. NHD may be the prototype of primary microglial disorders of the CNS or, as they have been coined, "microgliopathies". Mutations in TREM2 and TYROBP genes are known to cause NHD. Interestingly, recent evidence-associated rare genetic variants of TREM2 gene with increased risk of Alzheimer's disease, frontotemporal dementia, amyotrophic lateral sclerosis, and Parkinson's disease...
January 20, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28213912/diagnostic-red-flags-steroid-treated-malignant-cns-lymphoma-mimicking-autoimmune-inflammatory-demyelination
#3
Alonso Barrantes-Freer, Aylin Sophie Engel, Odir Antonio Rodríguez-Villagra, Anne Winkler, Markus Bergmann, Christian Mawrin, Tania Kuempfel, Hannah Pellkofer, Imke Metz, Annalen Bleckmann, Silvia Hernández-Durán, Sven Schippling, Elisabeth J Rushing, Stephan Frank, Markus Glatzel, Jakob Matschke, Christian Hartmann, Guido Reifenberger, Wolf Müller, Hans-Ulrich Schildhaus, Wolfgang Brück, Christine Stadelmann
The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells...
February 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28213800/invasive-aspergillus-laryngotracheobronchitis-in-an-adult-with-primary-cns-lymphoma
#4
Mark E Barry, William Walsh Thomas, Buer Song, Natasha Mirza
Invasive aspergillosis can be difficult to diagnose, and early recognition and initiation of therapy is imperative for improving patient outcomes. A case of invasive Aspergillus laryngotracheobronchitis is presented here with a review of the relevant literature. A 58-year-old male undergoing treatment for CNS lymphoma presented with neutropenic sepsis and acute respiratory distress requiring intubation. Following extubation, he reported persistent hoarseness for 1-month duration and he was found to have pseudomembranous plaques and ulcers of the larynx, trachea, and right mainstem bronchus consistent with Aspergillus laryngotracheobronchitis...
February 17, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28213522/type-i-interferon-regulated-gene-expression-and-signaling-in-murine-mixed-glial-cells-lacking-signal-transducers-and-activators-of-transcription-1-or-2-or-interferon-regulatory-factor-9
#5
Wen Li, Markus J Hofer, Pattama Songkhunawej, So Ri Jung, Dale Hancock, Gareth Denyer, Iain L Campbell
Type I interferons (IFN-I) are critical in antimicrobial and antitumor defense. Although IFN-I signal via the interferon-stimulated gene factor 3 (ISGF3) complex consisting of STAT1, STAT2 and IRF9, IFN-I can mediate significant biological effects via ISGF3-independent pathways. For example, absence of STAT1, STAT2 or IRF9 exacerbates neurological disease in transgenic mice with CNS-production of IFN-Ι. Here we determined the role of IFN-I-driven, ISGF3-independent signaling in regulating global gene expression in STAT1, STAT2 or IRF9-deficient murine mixed glial cell cultures (MGCs)...
February 17, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28213370/promiscuity-among-the-mraps
#6
Adrian J L Clark, Li F Chan
The melanocortin 2 receptor accessory protein (MRAP) was originally discovered to be an essential co-receptor for the ACTH receptor/melanocortin 2 receptor, and it physically interacts with this receptor and is required for receptor trafficking and ligand binding. A related molecule, MRAP2, is mainly expressed in the CNS and appears to have a role with the melanocortin 4 receptor. Consistent with this is the observation that a massively obese phenotype develops when the Mrap2 gene is deleted in mice. However, the characteristics of this phenotype differ from those of Mc4r deleted mice, and suggest that an additional role, possibly resulting from an interaction with other receptors is possible...
February 17, 2017: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/28213183/a-role-for-the-serotonin-reuptake-transporter-in-the-brain-and-intestinal-features-of-autism-spectrum-disorders-and-developmental-antidepressant-exposure
#7
REVIEW
Kara Gross Margolis
Many disease conditions considered CNS-predominant harbor significant intestinal comorbidities. Serotonin (5-HT) and the serotonin reuptake transporter (SERT) have increasingly been shown to play important roles in both brain and intestinal development and long-term function. 5-HT and SERT may thus modulate critical functions in the development and perpetuation of brain-gut axis disease. We discuss the potential roles of 5-HT and SERT in the brain and intestinal manifestations of autism spectrum disorders and developmental antidepressant exposure...
February 14, 2017: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/28212849/hydrogen-sulfide-endoplasmic-reticulum-stress-and-alcohol-mediated-neurotoxicity
#8
REVIEW
Akash K George, Jyotirmaya Behera, Kimberly E Kelly, Yuankun Zhai, Neetu Tyagi
Alcohol is one of the most socially accepted addictive drugs in modern society. Its abuse affects virtually all organ systems with the central nervous system (CNS) being particularly vulnerable to excessive alcohol exposure. Alcohol exposure also causes profound damage to both the adult and developing brain. Excessive alcohol consumption induces numerous pathophysiological stress responses, one of which is the endoplasmic reticulum (ER) stress response. Potential mechanisms that trigger the alcohol induced ER stress response are either directly or indirectly related to alcohol metabolism, which include toxic levels of acetaldehyde and homocysteine, oxidative stress and abnormal epigenetic modifications...
February 14, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28212704/neurochemical-differences-in-learning-and-memory-paradigms-among-rats-supplemented-with-anthocyanin-rich-blueberry-diets-and-exposed-to-acute-doses-of-56-fe-particles
#9
Shibu M Poulose, Bernard M Rabin, Donna F Bielinski, Megan E Kelly, Marshall G Miller, Nopporn Thanthaeng, Barbara Shukitt-Hale
The protective effects of anthocyanin-rich blueberries (BB) on brain health are well documented and are particularly important under conditions of high oxidative stress, which can lead to "accelerated aging." One such scenario is exposure to space radiation, consisting of high-energy and -charge particles (HZE), which are known to cause cognitive dysfunction and deleterious neurochemical alterations. We recently tested the behavioral and neurochemical effects of acute exposure to HZE particles such as (56)Fe, within 24-48h after exposure, and found that radiation primarily affects memory and not learning...
February 2017: Life Sciences in Space Research
https://www.readbyqxmd.com/read/28212424/risk-factors-for-central-nervous-system-tumors-in-children-new-findings-from-a-case-control-study
#10
Rebeca Ramis, Ibon Tamayo-Uria, Diana Gómez-Barroso, Gonzalo López-Abente, Antonio Morales-Piga, Elena Pardo Romaguera, Nuria Aragonés, Javier García-Pérez
BACKGROUND: Central nervous system tumors (CNS) are the most frequent solid tumor in children. Causes of CNS tumors are mainly unknown and only 5% of the cases can be explained by genetic predisposition. We studied the effects of environmental exposure on the incidence of CNS tumors in children by subtype, according to exposure to industrial and/or urban environment, exposure to crops and according to socio-economic status of the child. METHODS: We carried out a population-based case-control study of CNS tumors in Spain, covering 714 incident cases collected from the Spanish Registry of Childhood Tumors (period 1996-2011) and 4284 controls, individually matched by year of birth, sex, and autonomous region of residence...
2017: PloS One
https://www.readbyqxmd.com/read/28212304/methylglyoxal-derived-advanced-glycation-endproducts-in-multiple-sclerosis
#11
Suzan Wetzels, Kristiaan Wouters, Casper G Schalkwijk, Tim Vanmierlo, Jerome J A Hendriks
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS). The activation of inflammatory cells is crucial for the development of MS and is shown to induce intracellular glycolytic metabolism in pro-inflammatory microglia and macrophages, as well as CNS-resident astrocytes. Advanced glycation endproducts (AGEs) are stable endproducts formed by a reaction of the dicarbonyl compounds methylglyoxal (MGO) and glyoxal (GO) with amino acids in proteins, during glycolysis. This suggests that, in MS, MGO-derived AGEs are formed in glycolysis-driven cells...
February 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28211815/generation-and-characterization-of-knock-in-mouse-models-expressing-versions-of-huntingtin-with-either-an-n17-or-a-combined-polyq-and-proline-rich-region-deletion
#12
Emily A André, Elise M Braatz, Jeh-Ping Liu, Scott O Zeitlin
BACKGROUND: The polyglutamine (polyQ) stretch of the Huntingtin protein (HTT) in mammals is flanked by a highly conserved 17 amino acid N-terminal domain (N17), and a proline-rich region (PRR). The PRR is a binding site for many HTT-interacting proteins, and the N17 domain regulates several normal HTT functions, including HTT's ability to associate with membranes and organelles. OBJECTIVE: This study investigates the consequence of deleting mouse Huntingtin's (Htt's) N17 domain or a combination of its polyQ stretch and PRR (QP) on normal Htt function in mice...
February 16, 2017: Journal of Huntington's Disease
https://www.readbyqxmd.com/read/28210215/tryptanthrin-suppresses-the-activation-of-the-lps-treated-bv2-microglial-cell-line-via-nrf2-ho-1-antioxidant-signaling
#13
Young-Won Kwon, So Yeong Cheon, Sung Yun Park, Juhyun Song, Ju-Hee Lee
Microglia are the resident macrophages in the central nervous system (CNS) and play essential roles in neuronal homeostasis and neuroinflammatory pathologies. Recently, microglia have been shown to contribute decisively to neuropathologic processes after ischemic stroke. Furthermore, natural compounds have been reported to attenuate inflammation and pathologies associated with neuroinflammation. Tryptanthrin (indolo[2,1-b]quinazoline-6,12-dione) is a phytoalkaloid with known anti-inflammatory effects in cells...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28210207/cannabinoid-receptor-2-signaling-in-neurodegenerative-disorders-from-pathogenesis-to-a-promising-therapeutic-target
#14
REVIEW
Tommaso Cassano, Silvio Calcagnini, Lorenzo Pace, Federico De Marco, Adele Romano, Silvana Gaetani
As a consequence of an increasingly aging population, the number of people affected by neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease and Huntington's disease, is rapidly increasing. Although the etiology of these diseases has not been completely defined, common molecular mechanisms including neuroinflammation, excitotoxicity and mitochondrial dysfunction have been confirmed and can be targeted therapeutically. Moreover, recent studies have shown that endogenous cannabinoid signaling plays a number of modulatory roles throughout the central nervous system (CNS), including the neuroinflammation and neurogenesis...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28210205/the-atp-gated-p2x7-receptor-as-a-target-for-the-treatment-of-drug-resistant-epilepsy
#15
REVIEW
Edward Beamer, Wolfgang Fischer, Tobias Engel
Despite the progress made in the development of new antiepileptic drugs (AEDs), the biggest challenges that epilepsy presents to drug development have remained unchanged for the last 80 years: finding a treatment with potential for modifying disease progression and reducing the percentage of patients resistant to all pharmacological interventions. The mechanism of action of the majority of AEDs is based on blocking Na(+) and/or Ca(2+) channels, promotion of GABA or inhibition of glutamate signaling. In order for further progress to be made, however, a fuller picture of epilepsy will need to be considered, including changes to blood-brain barrier permeability, synaptic plasticity, network reorganization, and gliosis...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28209182/mesenchymal-stem-cells-alleviate-japanese-encephalitis-virus-induced-neuroinflammation-and-mortality
#16
Peiyu Bian, Chuantao Ye, Xuyang Zheng, Jing Yang, Wei Ye, Yuan Wang, Yun Zhou, Hongwei Ma, Peijun Han, Hai Zhang, Ying Zhang, Fanglin Zhang, Yingfeng Lei, Zhansheng Jia
BACKGROUND: Japanese encephalitis virus (JEV) is the leading cause of viral encephalitis in Asia. Japanese encephalitis (JE) caused by JEV is characterized by extensive inflammatory cytokine secretion, microglia activation, blood-brain barrier (BBB) breakdown, and neuronal death, all of which contribute to the vicious cycle of inflammatory damage. There are currently no effective treatments for JE. Mesenchymal stem cells (MSCs) have been demonstrated to have a therapeutic effect on many central nervous system (CNS) diseases by regulating inflammation and other mechanisms...
February 16, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28208745/astrocytic-pathological-calcium-homeostasis-and-impaired-vesicle-trafficking-in-neurodegeneration
#17
REVIEW
Nina Vardjan, Alexej Verkhratsky, Robert Zorec
Although the central nervous system (CNS) consists of highly heterogeneous populations of neurones and glial cells, clustered into diverse anatomical regions with specific functions, there are some conditions, including alertness, awareness and attention that require simultaneous, coordinated and spatially homogeneous activity within a large area of the brain. During such events, the brain, representing only about two percent of body mass, but consuming one fifth of body glucose at rest, needs additional energy to be produced...
February 8, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28208701/excitotoxins-mitochondrial-and-redox-disturbances-in-multiple-sclerosis
#18
REVIEW
Cecilia Rajda, Dániel Pukoli, Zsuzsanna Bende, Zsófia Majláth, László Vécsei
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system (CNS). There is increasing evidence that MS is not only characterized by immune mediated inflammatory reactions, but also by neurodegenerative processes. There is cumulating evidence that neurodegenerative processes, for example mitochondrial dysfunction, oxidative stress, and glutamate (Glu) excitotoxicity, seem to play an important role in the pathogenesis of MS. The alteration of mitochondrial homeostasis leads to the formation of excitotoxins and redox disturbances...
February 8, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28208698/advances-in-molecular-imaging-of-locally-delivered-targeted-therapeutics-for-central-nervous-system-tumors
#19
REVIEW
Umberto Tosi, Christopher S Marnell, Raymond Chang, William C Cho, Richard Ting, Uday B Maachani, Mark M Souweidane
Thanks to the recent advances in the development of chemotherapeutics, the morbidity and mortality of many cancers has decreased significantly. However, compared to oncology in general, the field of neuro-oncology has lagged behind. While new molecularly targeted chemotherapeutics have emerged, the impermeability of the blood-brain barrier (BBB) renders systemic delivery of these clinical agents suboptimal. To circumvent the BBB, novel routes of administration are being applied in the clinic, ranging from intra-arterial infusion and direct infusion into the target tissue (convection enhanced delivery (CED)) to the use of focused ultrasound to temporarily disrupt the BBB...
February 8, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28208270/ceramic-based-multisite-platinum-microelectrode-arrays-morphological-characteristics-and-electrochemical-performance-for-extracellular-oxygen-measurements-in-brain-tissue
#20
Ana Ledo, Cátia F Lourenço, João Laranjinha, Christopher M A Brett, Greg A Gerhardt, Rui M Barbosa
Ceramic-based multisite Pt microelectrode arrays (MEAs) were characterized for their basic electrochemical characteristics and used for in vivo measurements of oxygen with high resolution in the brain extracellular space. The microelectrode array sites showed a very smooth surface mainly composed of thin-film polycrystalline Pt, with some apparent nanoscale roughness that was not translated into an increased electrochemical active surface area. The electrochemical cyclic voltammetric behavior was characteristic of bulk Pt in both acidic and neutral media...
February 7, 2017: Analytical Chemistry
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