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https://www.readbyqxmd.com/read/29625923/wilson-s-disease-a-2017-update
#1
Aurélia Poujois, France Woimant
Wilson's disease (WD) is characterised by a deleterious accumulation of copper in the liver and brain. It is one of those rare genetic disorders that benefits from effective and lifelong treatments that have dramatically transformed the prognosis of the disease. In Europe, its clinical prevalence is estimated at between 1.2 and 2/100,000 but the genetic prevalence is higher, at around 1/7000. Incomplete penetrance of the gene or the presence of modifier genes may account for the difference between the calculated genetic prevalence and the number of patients diagnosed with WD...
April 3, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29619652/six-indicator-genes-for-zinc-zn-homeostasis-in-freshwater-teleost-yellow-catfish-pelteobagrus-fulvidraco-molecular-characterization-mrna-tissue-expression-and-transcriptional-changes-to-zn-exposure
#2
Guang-Hui Chen, Zhi Luo, Chuan-Chuan Wei, Dan-Dan Li, Ya-Xiong Pan
Excessive Zn in the aquatic environment can be toxic and causes dysfunction in Zn homeostasis for fish, which ultimately influences the function of various biological processes. Zn homeostasis is controlled by Zn transporters. This study cloned and characterized the full-length cDNA sequences of six Zn transport-relevant genes (ZnT1, ZnT5, ZnT7, ZIP4, ZIP5 and MTF-1) from yellow catfish Pelteobagrus fulvidraco. The six genes share similar domains to their corresponding members of mammals. Their mRNA amounts were widely existent across eight tissues (intestine, liver, brain, heart, gill, muscle, spleen and mesenteric fat), but relatively predominant in the liver and intestine...
April 4, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/29616363/a-comparative-in-vivo-scrutiny-of-biosynthesized-copper-and-zinc-oxide-nanoparticles-by-intraperitoneal-and-intravenous-administration-routes-in-rats
#3
Ashajyothi C, Harish K Handral, Chandrakanth Kelmani R
During the present time, anti-microbial features of copper (Cu) and zinc oxide (ZnO) nanoparticles (NPs) are extensively used to combat the growth of pathogenic microbes. CuNPs and ZnONPs are recurrently used in cosmetics, medicine and food additives, and their potential for toxic impacts on human and ecosystem is of high concern. In this study, the fate and toxicity of 16- to 96-nm-ranged biosynthesized copper (Bio-CuNPs) and zinc oxide (Bio-ZnONPs) was assessed in male Wistar rats. In vivo exposures of the two nanoparticles are achieved through two different administration routes namely, intraperitoneal (i/p) and intravenous (i/v) injections...
April 3, 2018: Nanoscale Research Letters
https://www.readbyqxmd.com/read/29593127/sulfamethoxazole-induces-zinc-changes-at-hippocampal-mossy-fiber-synapses-from-pregnant-rats
#4
Vanessa N Corceiro, Fatima C Bastos, Carlos M Matias, Jose C Dionísio, Rosa M Santos, Luis M Rosario, Rosa M Quinta-Ferreira, M Emilia C Quinta-Ferreira
The accumulation of intracellular ionic zinc and pharmaceutical compounds, like the antibiotic sulfamethoxazole, may contribute to various neuropathologies. Sulfamethoxazole and the drug trimethoprim, are inhibitors of enzymes involved in the synthesis of tetrahydrofolate and also of carbonic anhydrases. The inhibition of the latter enzymes, which are localized both intra- and extracellularly and have a key role in pH regulation, causes alkalinization that is associated with higher spontaneous transmitter release...
March 2018: General Physiology and Biophysics
https://www.readbyqxmd.com/read/29589874/genome-editing-in-neuroepithelial-stem-cells-to-generate-human-neurons-with-high-adenosine-releasing-capacity
#5
Daniel Poppe, Jonas Doerr, Marion Schneider, Ruven Wilkens, Julius A Steinbeck, Julia Ladewig, Allison Tam, David E Paschon, Philip D Gregory, Andreas Reik, Christa E Müller, Philipp Koch, Oliver Brüstle
As a powerful regulator of cellular homeostasis and metabolism, adenosine is involved in diverse neurological processes including pain, cognition, and memory. Altered adenosine homeostasis has also been associated with several diseases such as depression, schizophrenia, or epilepsy. Based on its protective properties, adenosine has been considered as a potential therapeutic agent for various brain disorders. Since systemic application of adenosine is hampered by serious side effects such as vasodilatation and cardiac suppression, recent studies aim at improving local delivery by depots, pumps, or cell-based applications...
March 28, 2018: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29578075/the-effects-of-maternal-diabetes-and-insulin-treatment-on-neurogenesis-in-the-developing-hippocampus-of-male-rats
#6
Akram Sadeghi, Ebrahim Esfandiary, Javad Hami, Hossein Khanahmad, Zahra Hejazi, Mohammad Mardani, Shahnaz Razavi
Diabetes in pregnancy is associated with an increasing risk of congenital malformations and central nervous system disorders (CNS) especially hippocampal neuronal circuitry disruption as a discreet region involved in neurogenesis phenomenon. This study aimed to investigate the effect of maternal diabetes and insulin treatment on the expression and distribution pattern of NeuN and DCX as two important markers of neurogenesis paradigm in developing rat hippocampus. All animals were randomly divided into three groups as follows: Control group, Diabetic (STZ-D), Diabetic treated with insulin (STZ-INS)...
March 22, 2018: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/29575794/morphological-and-biochemical-features-of-cerebellar-cortex-after-exposure-to-zinc-oxide-nanoparticles-possible-protective-role-of-curcumin
#7
Mona G Amer, Rehab A Karam
Zinc oxide nanoparticles (ZnONPs) are widely used in the last decades. So, Investigation of its neurotoxic effect is important. This work aimed to investigate the potential adverse effects of ZnONPs on rat's cerebellar cortex and the possible neuroprotective role of curcumin. Forty male albino rats were randomly divided into control and experimental groups. Twenty rats were injected with ZnONPs and ten of them previously received curcumin before ZnONPs then continued together for 28 days. At the end of the experiment, cerebellar tissue samples were prepared for histological, morphometric, immunohistochemical study and tissue levels of oxidative stress markers and cytokine analysis...
March 25, 2018: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
https://www.readbyqxmd.com/read/29573576/toward-clinical-and-molecular-understanding-of-pathogenic-variants-in-the-zbtb18-gene
#8
Vyne van der Schoot, Sonja de Munnik, Hanka Venselaar, Mariet Elting, Grazia M S Mancini, Conny M A Ravenswaaij-Arts, Britt-Marie Anderlid, Han G Brunner, Servi J C Stevens
BACKGROUND: Patients with pathogenic variants in ZBTB18 present with Intellectual Disability (ID) with frequent co-occurrence of corpus callosum (CC) anomalies, hypotonia, microcephaly, growth problems and variable facial dysmorphologies. These features illustrate a key role for ZBTB18 in brain development. METHODS: Patients with a pathogenic variant in ZBTB18 were detected by diagnostic whole exome sequencing (WES) performed in our center. We reviewed the literature and used GeneMatcher to include other cases...
March 24, 2018: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29570807/panel-of-serum-protein-biomarkers-to-grade-the-severity-of-traumatic-brain-injury
#9
Raj Poovindran Anada, Kum Thong Wong, Jaime Jacqueline Jayapalan, Onn Haji Hashim, Dharmendra Ganesan
The Glasgow Coma Scale (GCS), which classifies patients into mild, moderate or severe traumatic brain injury (TBI), is a system used to prioritize treatment and prognosticate the severity of head injury. In this study, sera of patients with various stages of TBI, as well as control subjects, were analyzed to screen for proteins that may be used to complement the GCS system. By subjecting pooled serum samples to iTRAQ analysis for quantitative comparison of protein abundance, and attesting their altered levels using ELISA, we have detected increased levels of serum amyloid A, C-reactive protein, leucine-rich alpha-2-glycoprotein, lipopolysaccharide-binding protein, fibronectin, vitronectin and alpha-1-antichymotrypsin in patients across all strata of TBI relative to the controls...
March 23, 2018: Electrophoresis
https://www.readbyqxmd.com/read/29566716/overexpression-of-zinc-%C3%AE-2-glycoprotein-suppressed-seizures-and-seizure-related-neuroflammation-in-pentylenetetrazol-kindled-rats
#10
Ying Liu, Teng Wang, Xi Liu, Yuetao Wen, Tao Xu, Xinyuan Yu, Xin Wei, Xueying Ding, Lijuan Mo, Maojia Yin, Xinjie Tan, Lifen Chen
BACKGROUND: Zinc-α2-glycoprotein (ZAG) is a 42-kDa protein reported as an anti-inflammatory adipocytokine. Evidences from clinical and experimental studies revealed that brain inflammation plays important roles in epileptogenesis and seizure. Interestingly, closely relationship between ZAG and many important inflammatory mediators has been proven. Our previous study identified ZAG in neurons and found that ZAG is decreased in epilepsy and interacts with TGFβ and ERK. This study aimed to investigate the role of ZAG in seizure and explore its effect on seizure-related neuroinflammation...
March 22, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29562533/a-long-journey-into-aging-brain-aging-and-alzheimer-s-disease-following-the-oxidative-stress-tracks
#11
Patrizia Mecocci, Virginia Boccardi, Roberta Cecchetti, Patrizia Bastiani, Michela Scamosci, Carmelinda Ruggiero, Marta Baroni
The Editors of the Journal of Alzheimer's Disease invited Professor Patrizia Mecocci to contribute a review article focused on the importance and implications of her research on aging, brain aging, and senile dementias over the last years. This invitation was based on an assessment that she was one of the journal's top authors and a strong supporter of the concept that oxidative stress is a major contributor to several alterations observed in age-related conditions (sarcopenia, osteoporosis) and, more significantly, in brain aging suggesting a pivotal role in the pathogenesis and progression of one of the most dramatic age-related diseases, Alzheimer's disease (AD)...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29555950/zinc-ion-rapidly-induces-toxic-off-pathway-amyloid-%C3%AE-oligomers-distinct-from-amyloid-%C3%AE-derived-diffusible-ligands-in-alzheimer-s-disease
#12
Ming-Che Lee, Wan-Cheng Yu, Yao-Hsiang Shih, Chun-Yu Chen, Zhong-Hong Guo, Shing-Jong Huang, Jerry C C Chan, Yun-Ru Chen
Alzheimer's disease (AD) is the most prevalent neurodegenerative disease in the elderly. Zinc (Zn) ion interacts with the pathogenic hallmark, amyloid-β (Aβ), and is enriched in senile plaques in brain of AD patients. To understand Zn-chelated Aβ (ZnAβ) species, here we systematically characterized ZnAβ aggregates by incubating equimolar Aβ with Zn. We found ZnAβ40 and ZnAβ42 both form spherical oligomers with a diameter of ~12-14 nm composed of reduced β-sheet content. Oligomer assembly examined by analytical ultracentrifugation, hydrophobic exposure by BisANS spectra, and immunoreactivity of ZnAβ and Aβ derived diffusible ligands (ADDLs) are distinct...
March 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29553247/new-tools-uncover-new-functions-for-mobile-zinc-in-the-brain
#13
Jacob M Goldberg, Stephen J Lippard
No abstract text is available yet for this article.
March 19, 2018: Biochemistry
https://www.readbyqxmd.com/read/29548726/disruption-of-the-zinc-metabolism-in-rat-f%C3%A5-tal-brain-after-prenatal-exposure-to-cadmium
#14
Safa Ben Mimouna, Sana Boughammoura, Marouane Chemek, Zohra Haouas, Mohamed Banni, Imed Messaoudi
This study was carried out to investigate the effects of maternal Cd and/or Zn exposure on some parameters of Zn metabolism in fetal brain of Wistar rats. Thus, female controls and other exposed by the oral route during the gestation period to Cd (50 mg CdCl2 /L) and/or Zn (ZnCl2 60 mg/L) were used. The male fetuses at age 20 days of gestation (GD20) were sacrificed and their brains were taken for histological, chemical and molecular analysis. Zn depletion was observed in the brains of fetuses issued from mothers exposed to Cd...
March 13, 2018: Chemico-biological Interactions
https://www.readbyqxmd.com/read/29534157/the-striatal-kinase-dclk3-produces-neuroprotection-against-mutant-huntingtin
#15
Laurie Galvan, Laetitia Francelle, Marie-Claude Gaillard, Lucie de Longprez, Maria-Angeles Carrillo-de Sauvage, Géraldine Liot, Karine Cambon, Lev Stimmer, Sophie Luccantoni, Julien Flament, Julien Valette, Michel de Chaldée, Gwenaelle Auregan, Martine Guillermier, Charlène Joséphine, Fanny Petit, Caroline Jan, Margot Jarrige, Noëlle Dufour, Gilles Bonvento, Sandrine Humbert, Frédéric Saudou, Philippe Hantraye, Karine Merienne, Alexis-Pierre Bemelmans, Anselme L Perrier, Nicole Déglon, Emmanuel Brouillet
The neurobiological functions of a number of kinases expressed in the brain are unknown. Here, we report new findings on DCLK3 (doublecortin like kinase 3), which is preferentially expressed in neurons in the striatum and dentate gyrus. Its function has never been investigated. DCLK3 expression is markedly reduced in Huntington's disease. Recent data obtained in studies related to cancer suggest DCLK3 could have an anti-apoptotic effect. Thus, we hypothesized that early loss of DCLK3 in Huntington's disease may render striatal neurons more susceptible to mutant huntingtin (mHtt)...
March 9, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29525064/inhaled-methane-protects-rats-against-neurological-dysfunction-induced-by-cerebral-ischemia-and-reperfusion-injury-pi3k-akt-ho-1-pathway-involved
#16
Baocheng Zhang, Mingqiang Gao, Jie Shen, Daikun He
BACKGROUND AND AIMS: Cerebral ischemia and reperfusion (I/R) could produce excess reactive oxygen species (ROS), which in turn induce neurological dysfunction and inflammation in cerebral tissues. This study was designed to study the effect of methane on cerebral I/R injury. METHODS: Fifty Sprague-Dawley (SD) rats were used to induce an animal model of cerebral I/R injury. Methane was mixed with air to achieve a final concentration of 2.2%. Rats started to inhale methane-air mixture after ischemia and continued it during the reperfusion...
August 2017: Archives of Medical Research
https://www.readbyqxmd.com/read/29524138/purified-protein-delivery-to-activate-an-epigenetically-silenced-allele-in-mouse-brain
#17
Benjamin Pyles, Barbara J Bailus, Henriette O'Geen, David J Segal
The ability to activate or repress specific genes in the brain could have a tremendous impact for understanding and treating neurological disorders. Artificial transcription factors based on zinc finger, TALE, and CRISPR/Cas9 programmable DNA-binding platforms have been widely used to regulate the expression of specific genes in cultured cells, but their delivery into the brain represents a critical challenge to apply such tools in live animals. In previous work, we developed a purified, zinc finger-based artificial transcription factor that could be injected systemically, cross the blood-brain barrier, and alter expression of a specific gene in the brain of an adult mouse model of Angelman syndrome...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29522722/trim28-and-the-control-of-transposable-elements-in-the-brain
#18
REVIEW
Daniela A Grassi, Marie E Jönsson, Per Ludvik Brattås, Johan Jakobsson
TRIM28 is an epigenetic co-repressor protein that mediates transcriptional silencing. TRIM28 participates, together with the large family of Kruppel-associated box domain zinc finger proteins (KRAB-ZFP) transcription factors, in the repression of transposable elements (TE). Recent advances indicate that TRIM28-based repression of TEs occurs in the mammalian brain and may provide beneficial effects through the regulation of transcriptional networks. Here, we provide an overview of TRIM28-related functions, highlighting the role of controlling TEs in neural progenitor cells and discuss how this mechanism may have contributed to the evolution of the complex human brain...
March 6, 2018: Brain Research
https://www.readbyqxmd.com/read/29518786/analysis-of-long-noncoding-rna-and-mrna-expression-profiles-in-il-9-activated-astrocytes-and-eae-mice
#19
Xiaomei Liu, Qing Zhang, Weixiao Wang, Dongjiao Zuo, Jing Wang, Feng Zhou, Liping Niu, Xiangyang Li, Suping Qin, Yanbo Kou, Fanyun Kong, Wei Pan, Yugang Wang, Dianshuai Gao, Hong Sun, Jessica M Meves, Kuiyang Zheng, Renxian Tang
BACKGROUND/AIMS: Multiple sclerosis (MS) is an autoimmune disease in the central nervous system associated with demyelination and axonal injury. Astrocyte activation is involved in the pathogenesis of MS and experimental autoimmune encephalomyelitis (EAE), an animal model of MS. This study was designed to find potential lncRNAs in EAE mice and activated astrocytes. METHODS: we performed microarray analysis of lncRNAs from the brain tissues of EAE mice and primary mouse astrocytes treated with IL-9(50 ng/ml)...
March 2, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29517182/influence-of-selected-dietary-components-on-the-functioning-of-the-human-nervous-system
#20
Agnieszka Wendołowicz, Ewa Stefańska, Lucyna Ostrowska
The diet is directly connected not only with the physical status but also with the functioning of the brain and the mental status. The potentially beneficial nutrients with a protective effect on the nervous system function include amino acids (tryptophan, phenylalanine, tyrosine, taurine), glucose and vitamins C, E, D and beta-carotene, B group vitamins (vitamin B12, vitamin B6, vitamin B4, vitamin B1) and minerals (selenium, zinc, magnesium, sodium, iron, copper, manganese, iodine). The presence of antioxidants in the diet protects against oxidative damage to nervous system cells...
2018: Roczniki Państwowego Zakładu Higieny
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