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Shenghe Zhao, Jiangsheng Xie, Guanghui Cheng, Yuren Xiang, Houyu Zhu, Wenyue Guo, Han Wang, Minchao Qin, Xinhui Lu, Junle Qu, Jiannong Wang, Jianbin Xu, Keyou Yan
Hybrid perovskite thin films are prone to producing surface vacancies during the film formation, which degrade the stability and photovoltaic performance. Passivation via post-treatment can heal these defects, but present methods are slightly destructive to the bulk of 3D perovskite due to the solvent effect, which hinders fabrication reproducibility. Herein, nondestructive surface/interface passivation using 4-fluoroaniline (FAL) is established. FAL is not only an effective antisolvent candidate for surface modification, but also a large dipole molecule (2...
November 12, 2018: Small
Keith Crosby, Anthony M Crown, Brittany L Roberts, Hilda Brown, Jacob I Ayers, David R Borchelt
Mutations in superoxide dismutase 1 (SOD1) associated with familial amyotrophic lateral sclerosis (fALS) induce the protein to misfold and aggregate. Missense mutations at more than 80 different amino acid positions have been associated with disease. How these mutations heighten the propensity of SOD1 to misfold and aggregate is unclear. With so many mutations, it is possible that more than one mechanism of aggregation may be involved. Of many possible mechanisms to explain heightened aggregation, one that has been suggested is that mutations that eliminate charged amino acids could diminish repulsive forces that would inhibit aberrant protein:protein interactions...
2018: PloS One
Neetu Soni, Sunil Kumar, Karthika Srindharan, Prabhakar Mishra, Nishant Gupta, Girish Bathla, Jyantee Kalita, Sanjay Behari
PURPOSE: To differentiate intra-axial tuberculomas (TB) from metastases based on quantitative differences in the perfusion and diffusion indices of lesion and perilesional edema using arterial spin labeling (ASL) and diffusion tensor imaging (DTI) techniques. MATERIALS AND METHODS: This prospective study included newly diagnosed untreated 12 patients of TB and 13 of metastases who underwent routine magnetic resonance imaging including DTI and ASL sequences. A region of interest analysis was performed and cerebral blood flow (CBF) values of lesion (L), perilesional edema (PE), and normal contralateral white matter (CWM) were calculated...
October 9, 2018: Current Problems in Diagnostic Radiology
E Srinivasan, R Rajasekaran
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that has been associated with mutations in metalloenzyme superoxide dismutase (SOD1) causing protein structural destabilization and aggregation. However, the mechanistic action and the cure for the disease still remain obscure. Herein, we initially studied the conformational preferences of SOD1 protein structures upon substitution of Ala at Gly93 in comparison with that of wild type. Our results corroborated with the previous experimental studies on the aggregation and the destabilizing activity of mutant SOD1 protein G93A...
October 28, 2018: Journal of Computer-aided Molecular Design
Hiroya Naruse, Hiroyuki Ishiura, Jun Mitsui, Yuji Takahashi, Takashi Matsukawa, Masaki Tanaka, Koichiro Doi, Jun Yoshimura, Shinichi Morishita, Jun Goto, Tatsushi Toda, Shoji Tsuji
OBJECTIVES: To evaluate the burden of rare variants in the causative genes for amyotrophic lateral sclerosis (ALS) on the age at onset of ALS in a Japanese case series. METHODS: We conducted whole-exome sequencing analysis of 89 families with familial ALS (FALS) and 410 patients with sporadic ALS (SALS) to identify known pathogenic mutations or rare functionally predicted deleterious variants in the causative genes for ALS. Rare variants (minor allele frequency <1%) with scaled Combined Annotation-Dependent Depletion score >20 were defined as rare functionally predicted deleterious variants...
October 24, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
Edward Pokrishevsky, Luke McAlary, Natalie E Farrawell, Beibei Zhao, Mine Sher, Justin J Yerbury, Neil R Cashman
Over 160 mutations in superoxide dismutase 1 (SOD1) are associated with familial amyotrophic lateral sclerosis (fALS), where the main pathological feature is deposition of SOD1 into proteinaceous cytoplasmic inclusions. We previously showed that the tryptophan residue at position 32 (W32) mediates the prion-like propagation of SOD1 misfolding in cells, and that a W32S substitution blocks this phenomenon. Here, we used in vitro protein assays to demonstrate that a W32S substitution in SOD1-fALS mutants significantly diminishes their propensity to aggregate whilst paradoxically decreasing protein stability...
October 22, 2018: Scientific Reports
David Brenner, Kathrin Müller, Regina Gastl, Martin Gorges, Markus Otto, Elmar H Pinkhardt, Jan Kassubek, Jochen H Weishaupt, Albert C Ludolph
Intermediate-length ATXN2 CAG repeats are a risk factor for amyotrophic lateral sclerosis (ALS). Here we report on a female patient with heterozygous repeat expansion mutation in the CACNA1A gene presenting with a pure ALS syndrome while her father, who also carries that CACNA1A mutation, suffers from a classical spinocerebellar ataxia type 6. Hypothesizing that CACNA1A CAG repeat expansions could be a monogenic cause for familial ALS (fALS), we analyzed the CAG repeat lengths in CACNA1A in a large cohort of genetically unexplained patients with fALS...
September 22, 2018: Neurobiology of Aging
Bin Qi, Xin Li, Liang Sun, Bo Chen, Hao Chen, Chenchen Wu, Haibo Zhang, Xiaohai Zhou
Supramolecular nano-assemblies with tunable morphology have attracted extensive attention in composite material manufacturing and many other fields. Herein, a new class of shape-controlled dodecaborate-based supramolecular organic frameworks (BOFs), decorated with diverse ultrafine noble metal clusters (Au, Pd, Pt, Ag), has been successfully fabricated via the tunable host-guest assembly of cucurbit[n = 5,6,7,8]uril and Cs2[closo-B12H12]. Due to the unique dodecaborate-cucurbit[n]uril chaotropic effect, a breakthrough has been made in modulating the supramolecular frameworks in the urchin-like, network-like or octahedron-like structures without tedious chemical modifications or additional additives...
November 1, 2018: Nanoscale
Mehdi Elsayed Moussa, Martin Piesch, Martin Fleischmann, Andrea Schreiner, Michael Seidl, Manfred Scheer
The synthesis of the air-stable and highly soluble Cu(i)-acetonitrile salts [Cu(CH3CN)3.5][FAl] (1) ([FAl] = FAl{OC(C6F10)(C6F5)}3) and [Cu(CH3CN)4][TEF] (2) ([TEF] = Al{OC(CF3)3}4) is presented. Compound 1 reacts with the organometallic polyphosphorus complexes [Cp2Mo2(CO)4(η2-P2)] (A) and [(Cp*Fe(η5-P5)] (B) and salt 2 reacts with B to form one new (3) and three unprecedented (4-6) phosphorus-rich Cu(i) dimers with the general formulas [Cu2(μ,η1:η1-A)2(η2-A)2][FAl]2 (3), [Cu2(μ,η1:η1-A)2(η1-CH3CN)4][FAl]2 (4), [Cu2(μ,η1:η1-B)2(η1-CH3CN)4][FAl]2 (5) and [Cu2(μ,η1:η1-B)2(η1-CH3CN)4][TEF]2 (6)...
October 15, 2018: Dalton Transactions: An International Journal of Inorganic Chemistry
Francesca Massenzio, Emiliano Peña-Altamira, Sabrina Petralla, Marco Virgili, Giampaolo Zuccheri, Andrea Miti, Elisabetta Polazzi, Ilaria Mengoni, Deborah Piffaretti, Barbara Monti
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease. Mutations in the gene encoding copper/zinc superoxide dismutase-1 (SOD1) are responsible for most familiar cases, but the role of mutant SOD1 protein dysfunction in non-cell autonomous neurodegeneration, especially in relation to microglial activation, is still unclear. Here, we focused our study on microglial cells, which release SOD1 also through exosomes. We observed that in rat primary microglia the overexpression of the most-common SOD1 mutations linked to fALS (G93A and A4V) leads to SOD1 intracellular accumulation, which correlates to autophagy dysfunction and microglial activation...
October 10, 2018: Biochimica et biophysica acta. Molecular basis of disease
Naoshi Nishida, Takafumi Nishimura, Toshimi Kaido, Kosuke Minaga, Kentaro Yamao, Ken Kamata, Mamoru Takenaka, Hiroshi Ida, Satoru Hagiwara, Yasunori Minami, Toshiharu Sakurai, Tomohiro Watanabe, Masatoshi Kudo
Hepatocellular carcinoma (HCC) causes one of the most frequent cancer-related deaths; an HCC subset shows rapid progression that affects survival. We clarify molecular features of aggressive HCC, and establish a molecular scoring system that predicts metastasis after curative treatment. In total, 125 HCCs were examined for TP53 , CTNNB1 , and TERT promoter mutation, methylation of 8 tumor suppressor genes, and 3 repetitive DNA sequences to estimate promoter hypermethylation and global hypomethylation. A fractional allelic loss (FAL) was calculated to represent chromosomal instability through microsatellite analysis...
September 29, 2018: Cancers
E Srinivasan, R Rajasekaran
Mutation in Cu/Zn superoxide dismutase (SOD1) at position 85 from glycine to arginine was found to be a prominent cause of aggregation characterized by an increased content of β-sheets in familial amyotrophic lateral sclerosis (fALS). Various literatures reported that natural polyphenols could act as a β-sheet breaker and therefore, treated as a potential therapeutics against various aggregated proteins involved in neurodegenerative disorders. Through computational perspective, molecular docking, quantum chemical studies, and discrete molecular dynamics were implemented to study the binding and structural effect of natural polyphenols, kaempferol, and kaempferide on mutant SOD1...
September 2018: BioFactors
Shengxin Zhao, Zhonglin Chen, Binyuan Wang, Jimin Shen, Jinna Zhang, Dongmei Li
Remediation of high concentrations of Cr(VI) in wastewater involves its chemical reduction to Cr(III), a product with low toxicity that can be easily removed. To date, NaBH4 has rarely been used to reduce Cr(VI). This article reports a comparative study of Cr(VI) removal by NaBH4 and five sulfur-based reducing agents (FeSO4 , Na2 S2 O5 , NaHSO3 , Na2 S2 O3 , and Na2 SO3 ). The potential mechanisms of Cr(VI) removal by these six reducing agents with and without fly ash leachate (FAL) are also discussed. The results revealed that the reduction and subsequent removal of Cr(VI) are influenced by the hydrolysis and ionization of the reducing agents in solution...
December 2018: Chemosphere
Stefan Achermann, Per Falås, Adriano Joss, Cresten B Mansfeldt, Yujie Men, Bernadette Vogler, Kathrin Fenner
For many polar organic micropollutants, biotransformation by activated sludge microorganisms is a major removal process during wastewater treatment. However, our current understanding of how wastewater treatment operations influence microbial communities and their micropollutant biotransformation potential is limited, leaving major parts of observed variability in biotransformation rates across treatment facilities unexplained. Here, we present biotransformation rate constants for 42 micropollutants belonging to different chemical classes along a gradient of solids retention time (SRT)...
October 1, 2018: Environmental Science & Technology
Tommaso Iannitti, Joseph M Scarrott, Shibi Likhite, Ian R P Coldicott, Katherine E Lewis, Paul R Heath, Adrian Higginbottom, Monika A Myszczynska, Marta Milo, Guillaume M Hautbergue, Kathrin Meyer, Brian K Kaspar, Laura Ferraiuolo, Pamela J Shaw, Mimoun Azzouz
Of familial amyotrophic lateral sclerosis (fALS) cases, 20% are caused by mutations in the gene encoding human cytosolic Cu/Zn superoxide dismutase (hSOD1). Efficient translation of the therapeutic potential of RNAi for the treatment of SOD1-ALS patients requires the development of vectors that are free of significant off-target effects and with reliable biomarkers to discern sufficient target engagement and correct dosing. Using adeno-associated virus serotype 9 to deliver RNAi against hSOD1 in the SOD1G93A mouse model, we found that intrathecal injection of the therapeutic vector via the cisterna magna delayed onset of disease, decreased motor neuron death at end stage by up to 88%, and prolonged the median survival of SOD1G93A mice by up to 42%...
September 7, 2018: Molecular Therapy. Nucleic Acids
Andrzej M Fal, Dorota M Fal, Dorota Kiedik, Barbara Gad-Karpierz
No abstract text is available yet for this article.
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Ghanashyam D Ghadge, Brian K Kay, Claire Drigotas, Raymond P Roos
Mutations in Cu/Zn superoxide dismutase (SOD1) are the cause of ~20% of cases of familial ALS (FALS), which comprise ~10% of the overall total number of cases of ALS. Mutant (mt) SOD1 is thought to cause FALS through a gain and not loss in function, perhaps as a result of the mutant protein's misfolding and aggregation. Previously we used a phage display library to raise single chain variable fragment antibodies (scFvs) against SOD1, which were found to decrease aggregation of mtSOD1 and toxicity in vitro. In the present study, we show that two scFvs directed against SOD1 ameliorate disease in G93A mtSOD1 transgenic mice and also decrease motor neuron loss, microgliosis, astrocytosis, as well as SOD1 burden and aggregation...
August 31, 2018: Neurobiology of Disease
Avisek Majumder, Mahavir Singh, Akash K George, Jyotirmaya Behera, Neetu Tyagi, Suresh C Tyagi
Neoangiogenesis is a fundamental process which helps to meet energy requirements, tissue growth, and wound healing. Although previous studies showed that Peroxisome proliferator-activated receptor (PPAR-γ) regulates neoangiogenesis via upregulation of vascular endothelial growth factor (VEGF), and both VEGF and PPAR-γ expressions were inhibited during hyperhomocysteinemic (HHcy), whether these two processes could trigger pathological effects in skeletal muscle via compromising neoangiogenesis has not been studied yet...
September 2018: Physiological Reports
Joshua L Heuslein, Catherine M Gorick, Stephanie P McDonnell, Ji Song, Brian H Annex, Richard J Price
Arteriogenesis, the growth of endogenous collateral arteries bypassing arterial occlusion(s), is a fundamental shear stress-induced adaptation with implications for treating peripheral arterial disease (PAD). Nonetheless, endothelial mechano-signaling during arteriogenesis is incompletely understood. Here we tested the hypothesis that a mechanosensitive microRNA, miR-199a-5p, regulates perfusion recovery and collateral arteriogenesis following femoral arterial ligation (FAL) via control of monocyte recruitment and pro-arteriogenic gene expression...
September 7, 2018: Molecular Therapy. Nucleic Acids
Lihua He, Yan Zhao, Liying Xing, Pinggui Liu, Youwei Zhang, Zhiyong Wang
A low infrared emissivity coating was prepared using graphene surface-modified flaky aluminum complex powders (rGO-FAl) as fillers. The flaky aluminum powders were coated with graphene through chemical bonding. Compared with pure flaky aluminum, the Vis-NIR diffuse reflectance of rGO-FAl complex powders was significantly decreased, which was beneficial to the low glossiness of the coating. After the modification, the glossiness at 60° of the coating with 40% (mass fraction) pigments decreased from 12.8 to 6...
August 22, 2018: Materials
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