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Huan Zhang, Hui He, Xuemei Jiang, Zhining Xia, Weili Wei
Two dimensional (2D) transition metal dichalcogenides (TMDs) had attracted enormous interests owing to their extraordinary optical, physical and chemical properties. Herein, we prepared for the first time a series of chiral TMD QDs from MoS2 and WS2 bulk crystals by covalent modification with chiral ligands cysteine and penicillamine. The chiral TMD QDs were carefully investigated by spectroscopic and microscopic techniques. Their chiral optical activity was confirmed by distinct circular dichroism signals different to those of the chiral ligands...
August 16, 2018: ACS Applied Materials & Interfaces
Sanjeev K Bhoi, Suprava Naik, Jayantee Kalita, Usha K Misra
Lead poisoning following retained gunshot pellets resulting in recurrent episodes of encephalomyeloneuropathy is rare and association of intracranial aneurysm with lead poisoning is interesting. The case report describes about a 58-year ayurvedic doctor who was hospitalized for recurrent abdominal pain and limb weakness appearing spontaneously and improving in a few days to weeks. He had 20 such attacks in last 30 years starting at 25 years after the gunshot wound that resulted in retention of 2 lead pellets in his skull...
August 2, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yanling Hu, Yu He, Yaxue Han, Yili Ge, Gongwu Song, Jiangang Zhou
Stable copper nanoclusters (CuNCs) were prepared by utilizing D-penicillamine as both the stabilizer and reductant. The emission of the CuNCs (with excitation/emission peaks at 390/645 nm) is largely stabilized by coating with poly(sodium-p-styrenesulfonate) (PSS). Cytochrome c (Cyt c) quenches the fluorescence of the PSS-coated CuNCs, and this effect was exploited to design a quenchometric fluorometric assay for Cyt c. If trypsin is added to the loaded CuNCs, it will hydrolyze Cyt c to form peptide fragments, and fluorescence is gradually restored...
July 21, 2018: Mikrochimica Acta
Julia Wattacheril, Patrick R Shea, Saeed Mohammad, Cynthia Behling, Vimla Aggarwal, Laura A Wilson, Katherine P Yates, Joy Ito, Mark Fishbein, Nicholas Stong, Joel E Lavine, David B Goldstein
Diagnostic whole exome sequencing has proven highly successful in a range of rare diseases, particularly early onset genetic conditions. In more common conditions, however, exome sequencing for diagnostic purposes remains the exception. Here we describe a patient initially diagnosed with a common, complex liver disease, nonalcoholic fatty liver disease (NAFLD), who was determined to have Wilson Disease (WD) upon research-related exome sequencing. The patient presented as a 14.5-year-old adolescent with chronically elevated aminotransferases, normal ceruloplasmin, and histologic examination consistent with NAFLD with advanced fibrosis...
July 19, 2018: Cold Spring Harbor Molecular Case Studies
Charles E Mordaunt, Noreene M Shibata, Dorothy A Kieffer, Anna Czlonkowska, Tomasz Litwin, Karl H Weiss, Daniel N Gotthardt, Kristin Olson, Dongguang Wei, Stewart Cooper, Yu-Jui Yvonne Wan, Mohamed Ali, Janine M LaSalle, Valentina Medici
Wilson disease (WD) is caused by mutations in the copper transporter ATP7B, leading to copper accumulation in the liver and brain. Excess copper inhibits S-adenosyl-L-homocysteine hydrolase, leading to variable WD phenotypes from widespread alterations in DNA methylation and gene expression. Previously, we demonstrated that maternal choline supplementation in the Jackson toxic milk (tx-j) mouse model of WD corrected higher thioredoxin 1 transcript levels in fetal liver. Here, we investigated the effect of maternal choline supplementation on genome-wide DNA methylation patterns in tx-j fetal liver by whole-genome bisulfite sequencing (WGBS)...
July 16, 2018: Human Molecular Genetics
Tsu-Shing Wang, Cheng-Ping Lin, Yu-Pong Chen, Mu-Rong Chao, Chien-Chun Li, Kai-Li Liu
BACKGROUND: IARC has classified the betel nut as a human environmental carcinogen. Previous studies have found that arecoline (AR) is the major alkaloid present in the saliva of betel quid chewers. Saliva contains a large content of AR which has been further shown to cause mutation of oral mucosa cells, resulting in oral cancer. Whereas, to date, there are only few studies reported the hepatotoxicity associated with arecoline and betel nut chewing. Therefore, the main purpose of this study was to determine the toxic effects of AR and its oxidative metabolite, arecoline N-oxide (ARNO), in normal liver cell lines...
July 2, 2018: Environmental Toxicology
Ping Zhou, Huai-Sha Xu, Meng-Meng Li, Xiao-Dong Chen, Jun Wang, Hua-Bin Zhou, Ling Chen, Ning Zhang, Na Liu
Predators induce defensive responses and fear behaviours in prey. The rat exposure test (RET) is frequently used as an animal model of panic. Nitric oxide (NO) which has been reported to be activated by the NMDA receptor, in turn mediates calcium/calmodulin-dependent protein kinase II (CaMKII) signalling pathways in defensive responses. ACCN2, the orthologous human gene of acid-sensing ion channel 1a (ASIC1a), is also associated with panic disorder; however, few studies have focused on the role of ASIC1a in the modulation of panic and calcium/CaMKII signalling by NO...
November 1, 2018: Behavioural Brain Research
Justine Bonetti, Yi Zhou, Marianne Parent, Igor Clarot, Haiyan Yu, Isabelle Fries-Raeth, Pierre Leroy, Isabelle Lartaud, Caroline Gaucher
S-Nitrosothiols, a class of NO donors, demonstrate potential benefits for cardiovascular diseases. Drugs for such chronic diseases require long term administration preferentially through the oral route. However, the absorption of S-nitrosothiols by the intestine, which is the first limiting barrier for their vascular bioavailability, is rarely evaluated. Using an in vitro model of intestinal barrier, based on human cells, the present work aimed at elucidating the mechanisms of intestinal transport (passive or active, paracellular or transcellular pathway) and at predicting the absorption site of three S-nitrosothiols: S-nitrosoglutathione (GSNO), S-nitroso-N-acetyl-l-cysteine (NACNO) and S-nitroso-N-acetyl-d-penicillamine (SNAP)...
June 21, 2018: Biochemical Pharmacology
Mónica Zuzarte, Jorge M Alves-Silva, Melissa Alves, Carlos Cavaleiro, Lígia Salgueiro, Maria Teresa Cruz
ETHNOPHARMACOLOGICAL RELEVANCE: Thymus camphoratus and T. carnosus are widely used in Portugal for the treatment of inflammatory-related conditions, such as inflammation of the respiratory tract, being the later also used as an antitussive. AIM OF THE STUDY: Bearing in mind the lack of scientific studies focused on the pharmacological activity of Thymus camphoratus and T. carnosus, this work was designed to validate the anti-inflammatory properties ascribed to these traditional species and concomitantly to unveil both the putative molecular mechanisms behind their bioactivity as well as the safety profile of their essential oils and major compounds...
October 28, 2018: Journal of Ethnopharmacology
Jan Pfeiffenberger, Christine Marie Lohse, Daniel Gotthardt, Christian Rupp, Markus Weiler, Ulrike Teufel, Karl Heinz Weiss, Annika Gauss
OBJECTIVE: Urinary copper excretion rates and non-caeruloplasmin associated copper concentrations are increased in patients with Wilson disease. However, there is little literature describing the monitoring of these parameters over the long term. METHODS: This is a monocentric retrospective study including data collected between 2003 and 2015 from 321 patients with Wilson disease by chart review. The patients were under therapy with D-penicillamine, trientine, or zinc...
June 20, 2018: Journal of Inherited Metabolic Disease
Yun Zhang, Dongmei Wang, Wei Wei, Xuejun Zeng
BACKGROUND: Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation, particularly in the liver and brain. Systemic lupus erythematosus (SLE) is a multi-system disorder that can manifest in any system. Cases with concomitant WD and SLE, unrelated to treatment with penicillamine, have been rarely reported. CASE PRESENTATION: We report a case of a young woman who had typical neuropsychiatric symptoms and laboratory tests results of WD...
June 15, 2018: BMC Neurology
Jennifer-Christin Müller, Josef Lichtmannegger, Hans Zischka, Michael Sperling, Uwe Karst
Wilson disease (WD) is a rare genetic disorder of the copper metabolism leading to systemic copper accumulation, predominantly in the liver. The therapeutic approach in WD patients is the generation of a negative copper balance and the maintenance of copper homeostasis, currently by the use of copper chelators such as D-penicillamine (D-PA). However, in circumstances of delayed diagnosis, poor treatment compliance, or treatment failure, mortality is almost certain without hepatic transplantation. Moreover, even after years of D-PA treatment, high liver copper levels are present in WD patients...
September 2018: Journal of Trace Elements in Medicine and Biology
Yan Ping, Li Ruiyi, Yang Yongqiang, Li Zaijun, Gu Zhiguo, Wang Guangli, Liu Junkang
Pentaethylenehexamine and d-penicillamine co-functionalized graphene quantum dots (PEHA-GQD-DPA) was made via one two-step thermal pyrolysis. The resulting PEHA-GQD-DPA is composed of the graphene sheets with an average size of 3.16 nm and the rich of functional groups. It gives an ultra strong fluorescence emission with the fluorescent quantum yield of 90.91% and sensitive and selective optical response towards Hg2+ . The fluorescence intensity linearly decreases with the increase of Hg2+ in the range of 1...
October 5, 2018: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Misa Nishiura, Tatsuhiro Kojima, Asako Igashira-Kamiyama, Takumi Konno
A unique example of a ring-to-cage structural conversion in a multinuclear gold(I) coordination system with d-penicillamine (d-H2 pen) is reported. The reaction of [Au2 Cl2 (dppe)] (dppe=1,2-bis(diphenylphosphino)ethane) with d-H2 pen in a 1:1 ratio gave [Au4 (dppe)2 (d-pen)2 ] ([1]), in which two [Au2 (dppe)]2+ units are linked by two d-pen S atoms in a cyclic form so as to have two bidentate-N,O coordination arms. The subsequent reaction of [1] with Cu(OTf)2 afforded [Au4 Cu(dppe)2 (d-pen)2 ]2+ ([2]2+ ), in which a CuII ion is chelated by the two coordination arms in [1] to form an AuI 4 CuII bicyclic metallocage...
May 28, 2018: Chemistry, An Asian Journal
A Khelfi, M Azzouz, R Abtroun, M Reggabi, B Alamir
Toxic myopathies are a group of pathologies characterized by a structural and/or functional disturbance of muscles induced by an exogenous agent. The most frequent are those induced by drugs used in clinical practice. Illegal drugs, pesticides, solvents, metals and even physical and gaseous agents can cause this kind of disease and exert toxicity on muscle tissues. Some toxins from animals, plants or micro-organisms are potent myotoxic agents, which can lead to fatal complications. Respiratory arrest and rhabdomyolysis are often referred to as the ultimate complications of these pathologies...
July 2018: Annales Pharmaceutiques Françaises
Yantong Wan, Xiaoqin Jiang, Xuemei Lin
RATIONALE: Wilson's disease (WD), or hepatolenticular degeneration, is an autosomal recessive disorder with a prevalence of 1:50,000 to 1:100,000 live births. PATIENT CONCERNS: A 26-year-old primipara with WD was admitted to our hospital, due to awaiting delivery. Her main symptoms were slightly higher total bile acid (TBA) and bilateral depressed edema of lower limbs. DIAGNOSIS: She was at 38 weeks and 4 days of gestation with a 15-year history of WD, controlled with penicillamine in the past and replaced by zinc preparations from three months before pregnancy...
May 2018: Medicine (Baltimore)
Yuhei Yasukawa, Nobuto Yoshinari, Takumi Konno
Treatment of a racemic RhIII4ZnII4 complex, (Δ)4/(Λ)4-[Zn4O{Rh(aet)3}4]6+ (aet = 2-aminoethanethiolate), with d-penicillaminate (d-pen) gave optically pure ionic crystals composed of (Λ)4-[Zn4O{Rh(aet)3}4]6+ and ΛD-[Rh(d-pen)3]3-via intramolecular and intermolecular chiral transfers from d-pen.
May 10, 2018: Chemical Communications: Chem Comm
Rixing Zhan, Fan Wang, Ying Wu, Ying Wang, Wei Qian, Menglong Liu, Tengfei Liu, Weifeng He, Hui Ren, Gaoxing Luo
OBJECTIVE: Nitric oxide (NO) has emerged as a critical molecule in wound healing, but the mechanism underlying its activity is not well defined. Here, we explored the effect of NO on the de-adhesion of epidermal stem cells (ESCs) and the mechanism involved in this process. METHODS: The effects of NO on isolated human and mouse ESCs cultured in the presence of different concentrations of the NO donor S-nitroso-N-acetyl penicillamine (SNAP) were evaluated in cell de-adhesion assays mediated by integrin β and collagen IV...
April 23, 2018: Nitric Oxide: Biology and Chemistry
Yinglu Wang, Lin Han, Ning Yuan, Hanxuan Wang, Hongxing Li, Jinrong Liu, Huan Chen, Qiang Zhang, Suwei Dong
Peptidyl thioesters or their surrogates with C-terminal β-branched hydrophobic amino acid residues usually exhibit poor reactivities in ligation reactions. Thus, activation using exogenous additives is required to ensure an acceptable reaction efficiency. Herein, we report a traceless ligation at Val-Xaa sites under mild thiol additive-free reaction conditions, whereby the introduction of β-mercaptan on the C-terminal valine residue effectively activates the otherwise unreactive N -acyl-benzimidazolinone (Nbz), and enables the use of a one-pot ligation-desulfurization strategy to generate the desired peptide products...
February 21, 2018: Chemical Science
In Ho Lee, Yoon Jeong Lee, Hyobin Seo, Yi Seul Kim, Ju-Ock Nam, Byung-Duk Jeon, Tae-Dong Kwon
PURPOSE: This study aimed to examine the independent effect of electrical pulse stimulation(EPS) and nitric oxide(NO) on muscle contraction and their synergistic or combined effect on contraction phenomenon using C2C12 mouse skeletal muscle cells. METHODS: Some differentiated C2C12 myotube cells were untreated (control). Other cells did not receive EPS and did receive 0.5, 1.0, or 2.0 mM of the NO donor, S-nitroso-N-acetyl-penicillamine (SNAP; -E/S0.5, -E/S1.0, and -E/S2...
March 30, 2018: Journal of Exercise Nutrition & Biochemistry
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