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Bettina P Mihalas, Elizabeth G Bromfield, Jessie M Sutherland, Geoffry N De Iuliis, Eileen A McLaughlin, R John Aitken, Brett Nixon
An increase in oxidative protein damage is a leading contributor to age-associated decline in oocyte quality. By removing such damaged proteins, the proteasome plays an essential role in maintaining the fidelity of oocyte meiosis. In this study, we established that decreased proteasome activity in naturally aged, germinal vesicle (GV) mouse oocytes positively correlates with increased protein modification by the lipid aldehyde 4-hydroxynonenal (4-HNE). Further, attenuation of proteasome activity in GV oocytes of young animals was accompanied by an increase in 4-HNE-modified proteins, including α-tubulin, thereby contributing to a reduction in tubulin polymerization, microtubule stability, and integrity of oocyte meiosis...
October 10, 2018: Journal of Biological Chemistry
Brendan J Houston, Brett Nixon, Bruce V King, R John Aitken, Geoffry N De Iuliis
As the use of mobile phone devices is now highly prevalent, many studies have sought to evaluate the effects of the radiofrequency-electromagnetic radiation (RF-EMR) on both human health and biology. While several such studies have shown RF-EMR is capable of inducing cellular stress, the physicobiological origin of this stress remains largely unresolved. To explore the effect of RF-EMR on the male reproductive system, we exposed cultured mouse spermatogonial GC1 and spermatocyte GC2 cell lines, as well as cauda epididymal spermatozoa to a waveguide generating continuous wave RF-EMR (1...
2018: Frontiers in Public Health
Sophie Heissat, Amélie Harel, Khémary Um, Anne-Sophie Brunet, Valérie Hervieu, Olivier Guillaud, Jerome Dumortier, Alain Lachaux, Elisabeth Mintz, Muriel Bost
Wilson's disease (WD) is caused by mutations in the ATP7B gene responsible for a toxic copper overload mainly in the liver and the central nervous system. Phenotypic heterogeneity may challenge the diagnostic confirmation. Exchangeable copper (CuEXC) has recently been proposed as a new marker of WD, and its ratio to the total serum copper (Cus), Relative Exchangeable Copper (REC = CuEXC/Cus), as a diagnostic marker. This study aimed to investigate whether this could be confirmed in Atp7b-/- mice, an engineered WD animal model...
December 2018: Journal of Trace Elements in Medicine and Biology
Jitendra Pant, Jaya Sundaram, Marcus J Goudie, Dieu Thao Nguyen, Hitesh Handa
Open bone fractures are not only difficult to heal but also are at a high risk of infections. Annual cases of fractures which result from osteoporosis amount to approximately 9 million. Endogenously released nitric oxide (NO) has been shown to play a role in osteogenic differentiation in addition to eradicating infection against a wide variety of pathogens. In the current work, antimicrobial NO releasing 3D bone scaffolds were fabricated using S-nitroso-N-acetyl-penicillamine (SNAP) as the NO donor. During fabrication, nano-hydroxyapatite (nHA) was added to each of the scaffolds in the concentration range of 10-50 wt % in nHA-starch-alginate and nHA-starch-chitosan scaffolds...
September 19, 2018: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
Maryam Shahrajabian, Forough Ghasemi, M Reza Hormozi-Nezhad
The ability to recognize the molecular chirality of enantiomers is extremely important owing to their critical role in drug development and biochemistry. Convenient discrimination of enantiomers has remained a challenge due to lack of unsophisticated methods. In this work, we have reported a simple strategy for chiral recognition of thiol-containing amino acids including penicillamine (PA), and cysteine (Cys). We have successfully designed a nanoparticle-based chemiluminescence (CL) system based on the reaction between cadmium telluride quantum dots (CdTe QDs) and the enantiomers...
September 18, 2018: Scientific Reports
Chaojie Qu, Duobao Zhang, Ran Yang, Jingyu Hu, Lingbo Qu
In this work, a novel nitrogen and sulfur co-doped graphene quantum dots (N,S/GQDs) were synthesized by a simple and green pyrolysis method with citric acid as carbon source, d-Penicillamine as doped molecule. The obtained N,S/GQDs shows good water solubility, low cytotoxicity and high quantum yield of 56.4%. The doping of N and S can efficient promoted the coordination between the residual group of N,S/GQDs and Hg2+ , thus, the fluorescence of N,S/GQDs can be dramatically and selectively quenched by Hg2+ ...
August 2, 2018: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Sean P Hopkins, Megan C Frost
Polyvinyl chloride (PVC) is one of the most widely used polymers in medicine but has very poor biocompatibility when in contact with tissue or blood. To increase biocompatibility, controlled release of nitric oxide (NO) can be utilized to mitigate and reduce the inflammatory response. A synthetic route is described where PVC is aminated to a specified degree and then further modified by covalently linking S -nitroso- N -acetyl-d-penicillamine (SNAP) groups to the free primary amine sites to create a nitric oxide releasing polymer (SNAP-PVC)...
September 5, 2018: Bioengineering
Yonghua Chen, Bo Zhang, Shijian Cao, Wei Huang, Ni Liu, Wenming Yang
We aim to investigate the function and mechanism of GanDouLing combinated with Penicillamine on cerebrovascular injury in Wilson's disease (WD). ELISA was performed to analyze the expression of vascular injury factors. Pathological changes of cerebral vessels were observed by HE stain. Immunohistochemistry assays were performed to analyze the expression of ICAM-1, VCAM-1, and GRP78. Western blotting was measured to analyze the expression of caspase-3, caspase-12, PERK, eIF2α, and CHOP. Apoptosis was detected with TUNEL assay...
October 31, 2018: Bioscience Reports
Michail M Padrul, Anna A Olina, Gulnara K Sadykova
Wilson-Konovalov's disease (DWK) is a polysyndrome hereditary disease associated with excessive accumulation of copper due to the delay in its removal from the body. The condition of the reproductive system in patients with DWK is not well understood. It is proved that copper is a microelement necessary for the synthesis of estrogens, the secretion of prostaglandins in the endometrium. According to the results of the study, in reproductive age patients with DWK who do not receive adequate therapy, the reproductive function is impaired...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Maria A Montesu, Giuliana Onnis, Susanna Gunnella, Amelia Lissia, Rosanna Satta
BACKGROUND: Elastosis perforans serpiginosa (EPS) is an uncommon cutaneous disorder classified under perforating diseases (PD); a group of dermatoses with transepidermal extrusion of collagen or elastic tissue. Three EPS subtypes have been reported that differ according to aetiology, associated diseases, and histopathological features. Herein, we report a systematic review of the literature, as well as a case of a 41-year-old woman with Wilson disease treated with penicillamine (PCM), who developed EPS after 11 years of drug intake...
August 21, 2018: European Journal of Dermatology: EJD
Huan Zhang, Hui He, Xuemei Jiang, Zhining Xia, Weili Wei
Two-dimensional 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 quantum dots (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...
September 12, 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-yr-old adolescent with chronically elevated aminotransferases, normal ceruloplasmin, and histologic examination consistent with NAFLD with advanced fibrosis...
October 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...
October 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)...
September 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
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