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Lu Huang, Qi Lin, Yanxia Li, Guocai Zheng, Yiting Chen
Two new chiral membranes were prepared by modification of gold nanochannel membranes with D-penicillamine and N-acetyl-L-cysteine and were characterized by scanning electron microscopy and X-ray photoelectron spectroscopy. The effects of key factors such as the gold deposition time, the pH, and the concentration of sodium dihydrogen phosphate on the separation factor are discussed. Chiral resolution of amino acid enantiomers by the chiral membranes was investigated. The experimental results show that the D-penicillamine-modified membrane has good enantioselectivity toward tyrosine and phenylalanine enantiomers, whereas the N-acetyl-L-cysteine-modified membrane has good enantioselectivity toward tyrosine and tryptophan enantiomers...
November 19, 2018: Analytical and Bioanalytical Chemistry
Richard Seto, Lauren M Mathias, Andrew Kelly, Richard S Panush
We cared for a woman with sero-positive rheumatoid arthritis (RA), in clinical remission on oral methotrexate (MTX) and hydroxychloroquine, who wished to donate a kidney to a brother with end-stage renal disease (ESRD). We could find scant literature about this unusual clinical circumstance, and therefore review pertinent aspects of renal disease in RA, perioperative medical management, maintenance of disease remission, outcomes for RA patients who have donated kidneys, and relevant ethical issues. Renal complications in RA are not uncommon, with as many as 50% of patients at risk of reduced eGFR...
November 8, 2018: Clinical Rheumatology
Zahra Shojaeifard, Nahid Heidari, Bahram Hemmateenejad
Assays of ferric ion (Fe3+ ) with high sensitivity and selectivity have been required to evaluate its amount in environmental and biological systems. Herein, a novel fluorometric penicillamine-capped bimetallic gold-copper nanoclusters (PA-AuCu bi-MNCs) sensor was constructed for facile, environmentally friendly and quantitative detection of Fe3+ through inner filter effect (IFE) mechanism. One-step green synthetic approach was applied for the synthesis of AuCu bi-MNCs by using d-penicillamine (D-PA) as template and stabilizer...
February 15, 2019: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Jakob Damsgaard, Fin Stolze Larsen, Henriette Ytting
Wilson's disease (WD) is a genetic disorder resulting in accumulation of copper in the liver and other organs. When presenting as acute liver failure (WD-ALF), urgent orthotopic liver transplantation (OLT) is considered the only life-saving option. The revised WD prognostic index is highly accurate in predicting death due to WD-ALF (1,2). For a score ≥11 acute listing for OLT is recommended in European and American guidelines. Here we report a patient with WD-ALF and poor prognostic score that was rescued by high-volume plasma exchange (HVP)...
October 23, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Hongwei Ge, Kui Zhang, Huan Yu, Ji Yue, Long Yu, Xinfeng Chen, Tianxin Hou, Khalid A Alamry, Hadi M Marwani, Suhua Wang
This paper reported a dual-mode probe for D-penicillamine on the basis of pH-mediated gold nanoparticles aggregation and fluorescence resonance energy transfer (FRET) from carbon dots. D-penicillamine is a zwitterionic compound and has different forms depending on specific pH ranges. The thiol group of D-penicillamine has high affinity towards the surface of gold nanoparticles and can replace other surface ligands. When pH values were close to its isoelectrical point (pH(I)), the D-penicillamine capped gold nanoparticles aggregated through hydrogen bonding or electrostatic interactions, resulting in the releasing of carbon dots from gold nanoparticles...
November 2018: Journal of Fluorescence
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+ ...
January 5, 2019: 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 Heinz Weiss, Daniel N Gotthardt, Kristin Olson, Dongguang Wei, Stewart Cooper, Yu-Jui Yvonne Wan, Mohamed R 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 (TNX1) 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)...
November 15, 2018: Human Molecular Genetics
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