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https://www.readbyqxmd.com/read/29149772/natural-history-of-infantile-onset-spinal-muscular-atrophy
#1
Stephen J Kolb, Christopher S Coffey, Jon W Yankey, Kristin Krosschell, W David Arnold, Seward B Rutkove, Kathryn J Swoboda, Sandra P Reyna, Ai Sakonju, Basil T Darras, Richard Shell, Nancy Kuntz, Diana Castro, Julie Parsons, Anne M Connolly, Claudia A Chiriboga, Craig McDonald, W Bryan Burnette, Klaus Werner, Mathula Thangarajh, Perry B Shieh, Erika Finanger, Merit E Cudkowicz, Michelle M McGovern, D Elizabeth McNeil, Richard Finkel, Susan T Iannaccone, Edward Kaye, Allison Kingsley, Samantha R Renusch, Vicki L McGovern, Xueqian Wang, Phillip G Zaworski, Thomas W Prior, Arthur H M Burghes, Amy Bartlett, John T Kissel
OBJECTIVE: Infantile-onset spinal muscular atrophy (SMA) is the most common genetic cause of infant mortality, typically resulting in death prior to age 2. Clinical trials in this population require an understanding of disease progression and identification of meaningful biomarkers to hasten therapeutic development and predict outcomes. METHODS: A longitudinal, multi-center, prospective natural history study enrolled 26 SMA infants, and 27 control infants less than six months of age...
November 17, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29149767/the-effects-of-sleep-on-the-neural-correlates-of-pattern-separation
#2
Christopher R Doxey, Cooper Hodges, Ty Bodily, Nathan M Muncy, C Brock Kirwan
Effective memory representations must be specific to prevent interference between episodes that may overlap in terms of place, time, or items present. Pattern separation, a computational process performed by the hippocampus, overcomes this interference by establishing non-overlapping memory representations. Although it is widely accepted that declarative memories are consolidated during sleep, the effects of sleep on pattern separation have yet to be elucidated. We used whole-brain, high-resolution functional neuroimaging to investigate the effects of sleep on a task that places high demands on pattern separation...
November 17, 2017: Hippocampus
https://www.readbyqxmd.com/read/29149746/habitat-mosaics-and-path-analysis-can-improve-biological-conservation-of-aquatic-biodiversity-in-ecosystems-with-low-head-dams
#3
Sean M Hitchman, Martha E Mather, Joseph M Smith, Jane S Fencl
Conserving native biodiversity depends on restoring functional habitats in the face of human-induced disturbances. Low-head dams are a ubiquitous human impact that degrades aquatic ecosystems worldwide. To improve our understanding of how low-head dams impact habitat and associated biodiversity, our research examined complex interactions among three spheres of the total environment. i.e., how low-head dams (anthroposphere) affect aquatic habitat (hydrosphere), and native biodiversity (biosphere) in streams and rivers...
November 14, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/29149730/divergence-of-insulin-superfamily-ligands-receptors-and-igf-binding-proteins-in-marine-versus-freshwater-stickleback-evidence-of-selection-in-known-and-novel-genes
#4
Tim Pellissier, Hend Al Nafea, Sara V Good
Three-spine stickleback (Gasterosteus aculeatus) is a teleost model for understanding genetic, physiological and morphological changes accompanying freshwater (FW) adaptation. There is growing evidence that the insulin superfamily plays important roles in traits involved in marine and FW adaptation. We performed a candidate gene analysis to look for evidence of selection on 33 insulin superfamily ligand-receptor genes and insulin-like growth factor binding proteins (Igfbp's) in stickleback. Using genotype data from 11 marine and 10 FW populations, we calculated the number of SNPs per site in regulatory and intronic regions, the number of synonymous and nonsynonymous mutations in coding regions, Wright's fixation index (Fst), and performed t-tests to identify SNPs with divergent genotype frequencies between marine/FW versus Atlantic/Pacific populations...
November 10, 2017: Comparative Biochemistry and Physiology. Part D, Genomics & Proteomics
https://www.readbyqxmd.com/read/29149694/a-high-brightness-probe-of-polymer-nanoparticles-for-biological-imaging
#5
Sirong Zhou, Jiarong Zhu, Yaping Li, Liheng Feng
Conjugated polymer nanoparticles (CPNs) with high brightness in long wavelength region were prepared by the nano-precipitation method. Based on fluorescence resonance energy transfer (FRET) mechanism, the high brightness property of the CPNs was realized by four different emission polymers. Dynamic light scattering (DLS) and scanning electron microscopy (SEM) displayed that the CPNs possessed a spherical structure and an average diameter of ~75nm. Analysis assays showed that the CPNs had excellent biocompatibility, good photostability and low cytotoxicity...
November 11, 2017: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
https://www.readbyqxmd.com/read/29149687/pyrrolidinyl-pna-polypyrrole-silver-nanofoam-electrode-as-a-novel-label-free-electrochemical-mirna-21-biosensor
#6
Tawatchai Kangkamano, Apon Numnuam, Warakorn Limbut, Proespichaya Kanatharana, Tirayut Vilaivan, Panote Thavarungkul
A label-free electrochemical miRNA biosensor was developed based on a pyrrolidinyl peptide nucleic acid (acpcPNA)/polypyrrole (PPy)/silver nanofoam (AgNF) modified electrode. The AgNF was electrodeposited as redox indicator on a gold electrode, which was then functionalized with an electropolymerized layer of PPy, a conducting polymer, to immobilize the PNA probes. The fabrication process was investigated by electrochemical impedance spectroscopy. The biosensor was used to detect miRNA-21, a biomarker abnormally expressed in most cancers...
November 6, 2017: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/29149660/a-systems-level-approach-for-investigating-organophosphorus-pesticide-toxicity
#7
Jingbo Zhu, Jing Wang, Yan Ding, Baoyue Liu, Wei Xiao
The full understanding of the single and joint toxicity of a variety of organophosphorus (OP) pesticides is still unavailable, because of the extreme complex mechanism of action. This study established a systems-level approach based on systems toxicology to investigate OP pesticide toxicity by incorporating ADME/T properties, protein prediction, and network and pathway analysis. The results showed that most OP pesticides are highly toxic according to the ADME/T parameters, and can interact with significant receptor proteins to cooperatively lead to various diseases by the established OP pesticide -protein and protein-disease networks...
November 14, 2017: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/29149617/decoding-early-and-late-cortical-contributions-to-individuation-of-attended-and-unattended-objects
#8
Claire K Naughtin, Jason B Mattingley, Angela D Bender, Paul E Dux
To isolate a visual stimulus as a unique object with a specific spatial location and time of occurrence, it is necessary to first register (individuate) the stimulus as a distinct perceptual entity. Recent investigations into the neural substrates of object individuation have suggested it is subserved by a distributed neural network, but previous manipulations of individuation load have introduced extraneous visual confounds, which might have yielded ambiguous findings, particularly in early cortical areas...
November 8, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29149546/the-photophysical-and-photoacoustic-properties-of-quadrupolar-borondifluoride-curcuminoid-dyes
#9
Stephanie Bellinger, Maryam Hatamimoslehabadi, Seema Bag, Farha Mithila, Jeffrey La, Mathieu Frennete, Samir Laoui, David Szalda, Chandra Yelleswarapu, Jonathan Rochford
The synthesis and characterization of a series of donor-π-acceptor-π-donor (D-A-D) curcuminoid molecules are here presented incorporating π-extended aryl and electron-donating amino terminal functionalization. Computational evaluation shows these molecules possess quadrupolar character with the lowest energy transitions displaying high molar extinction coefficients with broad tunability through manipulation of terminal donating groups. Consistent with their quadrupolar nature, these molecules show varying degrees of solvatochromic behavior in both their absorption and emission spectra which has been analyzed by Lippert-Mataga and Kamlet-Taft analysis...
November 17, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29149533/monocyte-specific-knockout-of-c-ebp%C3%AE-results-in-osteopetrosis-phenotype-blocks-bone-loss-in-ovariectomized-mice-and-reveals-an-important-function-of-c-ebp%C3%AE-in-osteoclast-differentiation-and-function
#10
Wei Chen, Guochun Zhu, Joel Jules, Diep Nguyen, Yi-Ping Li
CCAAT/enhancer-binding protein α (C/ebpα) is critical for osteoclastogenesis by regulating osteoclast (OC) lineage commitment and is also important for OC differentiation and function in vitro. However, the role of C/ebpα in postnatal skeletal development has not been reported owing to lethality in C/ebpα(-/-) mice from hypoglycemia within 8 hours after birth. Herein, we generated conditional knockout mice by deleting the C/ebpα gene in monocyte via LysM-Cre to examine its role in OC differentiation and function...
November 17, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29149510/transferrin-as-a-predictor-of-survival-in-cirrhosis
#11
André Viveiros, Armin Finkenstedt, Benedikt Schaefer, Mattias Mandorfer, Bernhard Scheiner, Konrad Lehner, Moritz Tobiasch, Thomas Reiberger, Herbert Tilg, Michael Edlinger, Heinz Zoller
BACKGROUND AND AIMS: Patients with cirrhosis frequently present with high serum ferritin and low transferrin concentrations, reflecting impaired liver function and inflammation. Recent studies have shown that transferrin and its saturation with iron are MELD-independent predictors of mortality in patients with acute on chronic liver failure or decompensated cirrhosis. The aim of this study was to evaluate the prognostic utility of serum iron parameters in relation to markers of liver function and immune activation...
November 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29149484/comparative-analysis-of-proteomes-between-diabetic-and-normal-human-sperm-insights-into-the-effects-of-diabetes-on-male-reproduction-based-on-the-regulation-of-mitochondria-related-proteins
#12
Tian An, Yue-Fen Wang, Jia-Xian Liu, Yan-Yun Pan, Yu-Fei Liu, Zhong-Chen He, Fang-Fang Mo, Jun Li, Li-Hua Kang, Yu-Jie Gu, Bo-Han Lv, Si-Huan Gao, Guang-Jian Jiang
This study sought to identify sources of the reduced fertility of men with type 2 diabetes mellitus. Significant reductions in semen volume, sperm concentration, and total sperm count were observed in diabetic individuals, while transmission electron microscopy revealed that the structure of mitochondria in the tail of sperm from diabetic patients was damaged. Proteins potentially associated with these sperm defects were identified using proteomics. Isobaric tagging for relative and absolute quantitation labeling and high-performance liquid chromatography-tandem mass spectrometry allowed us to identify 357 proteins significantly differentially expressed in diabetic versus control semen (>1...
November 17, 2017: Molecular Reproduction and Development
https://www.readbyqxmd.com/read/29149461/long-non-coding-rnas-involve-in-resistance-to-verticillium-dahliae-a-fungal-disease-in-cotton
#13
Lin Zhang, Maojun Wang, Nannan Li, Honglei Wang, Ping Qiu, Liuling Pei, Zheng Xu, Tianyi Wang, Erlin Gao, Junxia Liu, Shiming Liu, Qin Hu, Yuhuan Miao, Keith Lindsey, Lili Tu, Longfu Zhu, Xianlong Zhang
Long non-coding RNAs (lncRNAs) have several known functions in plant development, but their possible roles in responding to plant disease remain largely unresolved. In this study, we described a comprehensive disease-responding lncRNA profiles in defense against a cotton fungal disease Verticillium dahliae. We further revealed the conserved and specific characters of disease responding process between two cotton species. Conservatively for two cotton species, we found the expression dominance of induced lncRNAs in the Dt subgenome, indicating a biased induction pattern in the co-existing subgenomes of allotetraploid cotton...
November 17, 2017: Plant Biotechnology Journal
https://www.readbyqxmd.com/read/29149454/melatonin-alleviates-adipose-inflammation-through-elevating-%C3%AE-ketoglutarate-and-diverting-adipose-derived-exosomes-to-macrophages-in-mice
#14
Zhenjiang Liu, Lu Gan, Tiantian Zhang, Qian Ren, Chao Sun
Obesity is associated with macrophage infiltration and metabolic inflammation, both of which promote metabolic disease progression. Melatonin is reported to possess anti-inflammatory properties by inhibiting inflammatory response of adipocytes and macrophages activation. However, the effects of melatonin on the communication between adipocytes and macrophages during adipose inflammation remain elusive. Here, we demonstrated melatonin alleviated inflammation and elevated α-ketoglutarate (αKG) level in adipose tissue of obese mice...
November 17, 2017: Journal of Pineal Research
https://www.readbyqxmd.com/read/29149426/relationship-between-lat1-expression-and-resistance-to-chemotherapy-in-pancreatic-ductal-adenocarcinoma
#15
Bolag Altan, Kyoichi Kaira, Akira Watanabe, Norio Kubo, Pinjie Bao, Gantumur Dolgormaa, Erkhem-Ochir Bilguun, Kenichiro Araki, Yoshikatsu Kanai, Takehiko Yokobori, Tetsunari Oyama, Masahiko Nishiyama, Hiroyuki Kuwano, Ken Shirabe
PURPOSE: L-type amino acid transporter 1 (LAT1) is linked to tumor cell proliferation, angiogenesis, and survival in various human cancers. Although the expression of LAT1 was identified as a significant prognostic predictor after surgery in patients with pancreatic ductal adenocarcinoma (PDAC), little is known about the clinical significance of LAT1 as a chemotherapeutic resistance factor in PDAC. METHODS: A total of 110 patients with surgically resected PDAC were retrospectively reviewed as the training set...
November 17, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/29149417/spl13-regulates-shoot-branching-and-flowering-time-in-medicago-sativa
#16
Ruimin Gao, Margaret Y Gruber, Lisa Amyot, Abdelali Hannoufa
Our results show SPL13 plays a crucial role in regulating vegetative and reproductive development in Medicago sativa L. (alfalfa), and that MYB112 is targeted and downregulated by SPL13 in alfalfa. We previously showed that transgenic Medicago sativa (alfalfa) plants overexpressing microRNA156 (miR156) show a bushy phenotype, reduced internodal length, delayed flowering time, and enhanced biomass yield. In alfalfa, transcripts of seven SQUAMOSA-PROMOTER BINDING PROTEIN-LIKE (SPL) transcription factors, including SPL13, are targeted for cleavage by miR156...
November 17, 2017: Plant Molecular Biology
https://www.readbyqxmd.com/read/29149247/quantitative-proteomic-profiling-of-bovine-follicular-fluid-during-follicle-development
#17
Rodrigo de Andrade Ferrazza, Elizabeth Moreira Dos Santos Schmidt, Monika Mihm Carmichael, Fabiana Ferreira de Souza, Richard Burchmore, Roberto Sartori, Peter David Eckersall, João Carlos Pinheiro Ferreira
Bovine follicular fluid (FF) constitutes the microenvironment of follicles and includes various biologically active proteins. We performed a study involving 18 healthy non-lactating Holstein cows to determine the protein expression profile of FF at key-stages of follicular development. Follicles were individually aspirated in vivo at pre-deviation (F1∼7.0mm); deviation (F1∼8.5mm); post-deviation (F1∼12.0mm); and pre-ovulatory stages of follicle development, which were confirmed by measurement of follicular estradiol and progesterone concentrations...
November 15, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/29149236/mutations-affecting-glycinergic-neurotransmission-in-hyperekplexia-increase-pain-sensitivity
#18
Pascal Henri Vuilleumier, Raphael Fritsche, Jürg Schliessbach, Bernhard Schmitt, Lars Arendt-Nielsen, Hanns Ulrich Zeilhofer, Michele Curatolo
Inhibitory interneurons in the spinal cord use glycine and GABA for fast inhibitory neurotransmission. While there is abundant research on these inhibitory pain pathways in animal models, their relevance in humans remains unclear, largely due to the limited possibility to manipulate selectively these pathways in humans. Hyperekplexia is a rare human disease that is caused by loss-of-function mutations in genes encoding for glycine receptors and glycine transporters. In the present study, we tested whether hyperekplexia patients display altered pain perception or central pain modulation compared with healthy subjects...
November 15, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29149198/experiences-of-older-people-with-dementia-participating-in-a-high-intensity-functional-exercise-program-in-nursing-homes-while-it-s-tough-it-s-useful
#19
Nina Lindelöf, Lillemor Lundin-Olsson, Dawn A Skelton, Berit Lundman, Erik Rosendahl
The objective of the study was to describe the views and experiences of participation in a high-intensity functional exercise (HIFE) program among older people with dementia in nursing homes. The study design was a qualitative interview study with 21 participants (15 women), aged 74-96, and with a Mini-Mental State Examination score of 10-23 at study start. The HIFE-program comprises exercises performed in functional weight-bearing positions and including movements used in everyday tasks. The exercise was individually designed, supervised in small groups in the nursing homes and performed during four months...
2017: PloS One
https://www.readbyqxmd.com/read/29149177/new-brain-lesions-with-no-impact-on-physical-disability-can-impact-cognition-in-early-multiple-sclerosis-a-ten-year-longitudinal-study
#20
D Wybrecht, F Reuter, F Pariollaud, W Zaaraoui, A Le Troter, A Rico, S Confort-Gouny, E Soulier, M Guye, A Maarouf, J-P Ranjeva, J Pelletier, B Audoin
OBJECTIVE: In early multiple sclerosis, although brain T2 lesions accrual are hallmark of the disease, only weak correlations were found between T2 lesions accrual and EDSS progression, the disability scale commonly used in multiple sclerosis studies. This may be related to the very poor sensitivity of EDSS to cognitive dysfunctions that may occur and progress from the first stage of the disease. In the present study, we aimed to demonstrate that cognitive deficits progress during the first ten years of MS and are significantly impacted by new T2 lesions...
2017: PloS One
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