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https://www.readbyqxmd.com/read/27911337/effect-of-the-butyrate-prodrug-pivaloyloxymethyl-butyrate-an9-on-a-mouse-model-for-spinal-muscular-atrophy
#1
Jonathan D Edwards, Matthew E R Butchbach
Spinal muscular atrophy (SMA) is an early-onset motor neuron disease that leads to loss of muscle function. Butyrate (BA)-based compounds markedly improve the survival and motor phenotype of SMA mice. In this study, we examine the protective effects of the BA prodrug pivaloyloxymethyl butyrate (AN9) on the survival of SMNΔ7 SMA mice. Oral administration of AN9 beginning at PND04 almost doubled the average lifespan of SMNΔ7 SMA mice. AN9 treatment also increased the growth rate of SMNΔ7 SMA mice when compared to vehicle-treated SMNΔ7 SMA mice...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27911332/type-0-spinal-muscular-atrophy-further%C3%A2-delineation-of-prenatal-and%C3%A2-postnatal-features-in-16-patients
#2
Sarah Grotto, Jean-Marie Cuisset, Stéphane Marret, Séverine Drunat, Patricia Faure, Séverine Audebert-Bellanger, Isabelle Desguerre, Vincent Flurin, Anne-Gaëlle Grebille, Anne-Marie Guerrot, Hubert Journel, Gilles Morin, Ghislaine Plessis, Sylvain Renolleau, Joëlle Roume, Brigitte Simon-Bouy, Renaud Touraine, Marjolaine Willems, Thierry Frébourg, Eric Verspyck, Pascale Saugier-Veber
BACKGROUND: Spinal muscular atrophy (SMA) is caused by homozygous inactivation of the SMN1 gene. The SMN2 copy number modulates the severity of SMA. The 0SMN1/1SMN2 genotype, the most severe genotype compatible with life, is expected to be associated with the most severe form of the disease, called type 0 SMA, defined by prenatal onset. OBJECTIVE: The aim of the study was to review clinical features and prenatal manifestations in this rare SMA subtype. METHODS: SMA patients with the 0SMN1/1SMN2 genotype were retrospectively collected using the UMD-SMN1 France database...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27909747/transcranial-direct-current-stimulation-tdcs-to-the-supplementary-motor-area-sma-influences-performance-on-motor-tasks
#3
K E Hupfeld, C J Ketcham, H D Schneider
The supplementary motor area (SMA) is believed to be highly involved in the planning and execution of both simple and complex motor tasks. This study aimed to examine the role of the SMA in planning the movements required to complete reaction time, balance, and pegboard tasks using anodal transcranial direct current stimulation (tDCS), which passes a weak electrical current between two electrodes, in order to modulate neuronal activity. Twenty healthy adults were counterbalanced to receive either tDCS (experimental condition) or no tDCS (control condition) for 3 days...
December 1, 2016: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/27909731/hypoxia-drives-the-transition-of-human-dermal-fibroblasts-to-a-myofibroblast-like-phenotype-via-the-tgf-%C3%AE-1-smad3-pathway
#4
Bin Zhao, Hao Guan, Jia-Qi Liu, Zhao Zheng, Qin Zhou, Jian Zhang, Lin-Lin Su, Da-Hai Hu
Keloids, partially considered as benign tumors, are characterized by the overgrowth of fibrosis beyond the boundaries of the wound and are regulated mainly by transforming growth factor (TGF)-β1, which induces the transition of fibroblasts to myofibroblasts. Hypoxia is an important driving force in the development of lung and liver fibrosis by activating hypoxia inducible factor-1α and stimulating epithelial‑mesenchymal transition. However, it is unknown whether and hypoxia can influence human dermal scarring...
December 1, 2016: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/27909707/in-vitro-study-of-the-host-responses-to-model-biomaterials-via-a-fibroblast-macrophage-co-culture-system
#5
Guoying Zhou, Andrea Liedmann, Chandralekha Chatterjee, Thomas Groth
Surface properties are believed to play important roles in initial inflammatory and subsequent wound healing/fibrotic responses after implantation of biomaterials. To investigate the surface property effect in mediating these host responses, we used an in vitro fibroblast/macrophage co-culture model established with a cell migration chamber, and a series of self-assembling monolayers (SAMs) bearing different terminal groups as model surfaces to study the effect of surface properties on macrophage fusion, fibroblast attachment, spreading morphology, proliferation, outgrowth, as well as pro-(interleukin-6) and anti-(interleukin-10) inflammatory cytokine production, expression of ED-A fibronectin (FN) and alpha-smooth muscle actin (α-SMA)...
December 2, 2016: Biomaterials Science
https://www.readbyqxmd.com/read/27909403/cortical-sensorimotor-processing-of-painful-pressure-in-patients-with-chronic-lower-back-pain-an-optical-neuroimaging-study-using-fnirs
#6
Andrea Vrana, Michael L Meier, Sabina Hotz-Boendermaker, Barry K Humphreys, Felix Scholkmann
In this study we investigated sensorimotor processing of painful pressure stimulation on the lower back of patients with chronic lower back pain (CLBP) by using functional near-infrared spectroscopy (fNIRS) to measure changes in cerebral hemodynamics and oxygenation. The main objectives were whether patients with CLBP show different relative changes in oxy- and deoxyhemoglobin ([O2Hb] and [HHb]) in the supplementary motor area (SMA) and primary somatosensory cortex (S1) compared to healthy controls (HC). Twelve patients with CLBP (32 ± 6...
2016: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/27908803/pericytes-integral-components-of-adult-hematopoietic-stem-cell-niches
#7
REVIEW
D Sá da Bandeira, J Casamitjana, M Crisan
The interest in perivascular cells as a niche for adult hematopoietic stem cells (HSCs) is significantly growing. In the adult bone marrow (BM), perivascular cells and HSCs cohabit. Among perivascular cells, pericytes are precursors of mesenchymal stem/stromal cells (MSCs) that are capable of differentiating into osteoblasts, adipocytes and chondrocytes. In situ, pericytes are recognised by their localisation to the abluminal side of the blood vessel wall, closely associated with endothelial cells in combination with the expression of markers such as CD146, neural glial 2 (NG2), platelet derived growth factor receptor β (PDGFRβ), α-smooth muscle actin (α-SMA), nestin (Nes) and/or leptin receptor (LepR)...
November 28, 2016: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/27907179/correction-cervical-spinal-cord-atrophy-profile-in-adult-smn1-linked-sma
#8
Mohamed-Mounir El Mendili, Timothée Lenglet, Tanya Stojkovic, Anthony Behin, Raquel Guimarães-Costa, François Salachas, Vincent Meininger, Gaelle Bruneteau, Nadine Le Forestier, Pascal Laforêt, Stéphane Lehéricy, Habib Benali, Pierre-François Pradat
[This corrects the article DOI: 10.1371/journal.pone.0152439.].
2016: PloS One
https://www.readbyqxmd.com/read/27907033/normalization-of-patient-identified-plasma-biomarkers-in-smn%C3%AE-7-mice-following-postnatal-smn-restoration
#9
W David Arnold, Sandra Duque, Chitra C Iyer, Phillip Zaworski, Vicki L McGovern, Shannon J Taylor, Katharine M von Herrmann, Dione T Kobayashi, Karen S Chen, Stephen J Kolb, Sergey V Paushkin, Arthur H M Burghes
INTRODUCTION AND OBJECTIVE: Spinal muscular atrophy (SMA) is an autosomal recessive motor neuron disorder. SMA is caused by homozygous loss of the SMN1 gene and retention of the SMN2 gene resulting in reduced levels of full length SMN protein that are insufficient for motor neuron function. Various treatments that restore levels of SMN are currently in clinical trials and biomarkers are needed to determine the response to treatment. Here, we sought to investigate in SMA mice a set of plasma analytes, previously identified in patients with SMA to correlate with motor function...
2016: PloS One
https://www.readbyqxmd.com/read/27906172/inflammatory-macrophages-can-transdifferentiate-into-myofibroblasts-during-renal-fibrosis
#10
Xiao-Ming Meng, Shuang Wang, Xiao-Ru Huang, Chen Yang, Jun Xiao, Yang Zhang, Ka-Fai To, David J Nikolic-Paterson, Hui-Yao Lan
Myofibroblasts play a central role in renal fibrosis although the origin of these cells remains controversial. We recently reported that bone marrow-derived macrophages can give rise to myofibroblasts through macrophage to myofibroblast transition (MMT). However, several important issues remain to be addressed, including whether MMT occurs in human kidney disease and verification of the MMT process through lineage tracing. Biopsies from a cohort of 58 patients with various forms of kidney disease were examined for MMT cells that co-express macrophage (CD68) and myofibroblast (α-smooth muscle actin, α-SMA) markers...
December 1, 2016: Cell Death & Disease
https://www.readbyqxmd.com/read/27906099/p311-induces-the-transdifferentiation-of-epidermal-stem-cells-to-myofibroblast-like-cells-by-stimulating-transforming-growth-factor-%C3%AE-1-expression
#11
Haisheng Li, Zhihui Yao, Weifeng He, Hongyan Gao, Yang Bai, Sisi Yang, Lu Zhang, Rixing Zhan, Jianglin Tan, Junyi Zhou, Masao Takata, Jun Wu, Gaoxing Luo
BACKGROUND: Epithelial to mesenchymal transition, especially to myofibroblasts, plays an important role in wound healing, fibrosis, and carcinogenesis. Epidermal stem cells (EpSCs) are responsible for epidermal renewal and wound re-epithelialization. However, it remains unclear whether and how EpSCs transdifferentiate into myofibroblasts or myofibroblast-like cells (MFLCs). Here, we provide the first evidence showing that P311 induces EpSC to MFLC transdifferentiation (EpMyT) via TGFβ1/Smad signaling...
December 1, 2016: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/27905588/phase-specific-dispersion-of-functional-nanoparticles-in-soft-nanocomposites-resulting-in-enhanced-electromagnetic-screening-ability-dominated-by-absorption
#12
Aishwarya V Menon, Giridhar Madras, Suryasarathi Bose
The effect of phase specific localisation of MWNTs (multiwalled carbon nanotubes) and magnetic FeNi (iron-nickel) alloy particles on bulk electrical conductivity and electromagnetic (EM) wave attenuation was investigated in biphasic co-continuous blends of PVDF/SMA (polyvinylidene fluoride/styrene maleic anhydride). It is envisaged that packing different functional nanoparticles in a given phase of a co-continuous blend can impede the charge transport phenomenon and the overall dispersion state. Therefore, phase specific localisation can facilitate the tuning of the functional properties in biphasic blends...
December 1, 2016: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/27903487/disturbed-p53-mdm2-feedback-loop-contributes-to-thoracic-aortic-dissection-formation-and-may-be-a-result-of-trim-25-overexpression
#13
Bin Gong, Zhiwei Wang, Min Zhang, Zhipeng Hu, Zongli Ren, Zheng Tang, Wanli Jiang, Lianghao Cheng, Jun Huang, Wei Ren, Qingtao Wang
BACKGROUND: The development of thoracic aortic dissection (TAD) is attributed to a broad range of degenerative, genetic, structural, oxidative, apoptotic, and acquired disease states. In this study, we examined the role of the disturbed p53-MDM2 feed-back loop in the formation of TAD, and one of a potential feed-back loop regulator, TRIM-25. METHODS: Surgical specimens of the aorta from TAD patients (n=10) and controls (n=10) were tested for α-smooth muscle actin (α-SMA),p53,murine double minute2(MDM2) and tripartite motif protein-25(TRIM-25) by western blot, immunohistochemical staining and quantitative real-time reverse transcription polymerase chain reaction (qRT-PCR) , respectively...
November 26, 2016: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/27902771/piceatannol-attenuates-renal-fibrosis-induced-by-unilateral-ureteral-obstruction-via-downregulation-of-histone-deacetylase-4-5-or-p38-mapk-signaling
#14
Sin Young Choi, Zhe Hao Piao, Li Jin, Jung Ha Kim, Gwi Ran Kim, Yuhee Ryu, Ming Quan Lin, Hyung-Seok Kim, Hae Jin Kee, Myung Ho Jeong
Piceatannol, a resveratrol metabolite, is a phenolic compound found in red wine and grapes. We investigated the effect of piceatannol on renal fibrosis and histone deacetylase (HDAC) expression in a mouse model of unilateral ureteral obstruction (UUO). Fibrosis was established by UUO and piceatannol was intraperitoneally injected for 2 weeks. Piceatannol suppressed extracellular matrix (ECM) protein deposition including collagen type I and fibronectin as well as connective tissue growth factor (CTGF) and α-smooth muscle actin (α-SMA) in UUO kidneys...
2016: PloS One
https://www.readbyqxmd.com/read/27901488/cancer-associated-fibroblasts-promote-cancer-cell-growth-through-a-mir-7-rassf2-par-4-axis-in-the-tumor-microenvironment
#15
Zongze Shen, Xing Qin, Ming Yan, Rongrong Li, Gang Chen, Jianjun Zhang, Wantao Chen
Cancer-associated fibroblasts (CAFs), a major component of cancer stroma, play an important role in cancer progression but little is known about how CAFs affect tumorigenesis and development. MicroRNAs (miRNAs) are small non-coding RNAs that can negatively regulate target mRNA expression at post-transcriptional levels. In head and neck cancer (HNC), our analysis of miRNA arrays showed that miR-7, miR-196 and miR-335 were significantly up-regulated in CAFs when compared with their paired normal fibroblasts (NFs)...
November 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27901027/targeted-killing-of-myofibroblasts-by-biosurfactant-di-rhamnolipid-suggests-a-therapy-against-scar-formation
#16
Chong Shen, Lifang Jiang, Huawei Shao, Chuangang You, Guoliang Zhang, Sitong Ding, Tingwei Bian, Chunmao Han, Qin Meng
Pathological myofibroblasts are often involved in skin scarring via generating contractile force and over-expressing collagen fibers, but no compound has been found to inhibit the myofibroblasts without showing severe toxicity to surrounding physiological cells. Here we report that di-rhamnolipid, a biosurfactant secreted by Pseudomonas aeruginosa, showed potent effects on scar therapy via a unique mechanism of targeted killing the myofibroblasts. In cell culture, the fibroblasts-derived myofibroblasts were more sensitive to di-rhamnolipid toxicity than fibroblasts at a concentration-dependent manner, and could be completely inhibited of their specific functions including α-SMA expression and collagen secretion/contraction...
November 30, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27899769/the-role-of-club-cell-phenoconversion-and-migration-in-idiopathic-pulmonary-fibrosis
#17
Jutaro Fukumoto, Ramani Soundararajan, Joseph Leung, Ruan Cox, Sanjay Mahendrasah, Neha Muthavarapu, Travis Herrin, Alexander Czachor, Lee C Tan, Nima Hosseinian, Priyanshi Patel, Jayanthraj Gone, Mason T Breitzig, Young Cho, Andrew J Cooke, Lakshmi Galam, Venkata Ramireddy Narala, Yashwant Pathak, Richard F Lockey, Narasaiah Kolliputi
Idiopathic pulmonary fibrosis (IPF) is an age-related multifactorial disease featuring non-uniform lung fibrosis. The decisive cellular events at early stages of IPF are poorly understood. While the involvement of club cells in IPF pathogenesis is unclear, their migration has been associated with lung fibrosis. In this study, we labeled club cells immunohistochemically in IPF lungs using a club cell marker Claudin-10 (Cldn10), a unique protein based on the recent report which demonstrated that the appearance of Cldn10 in developing and repairing lungs precedes other club cell markers including club cell secretory protein (CCSP)...
November 29, 2016: Aging
https://www.readbyqxmd.com/read/27898661/methyl-cpg-binding-domain-3-inhibits-epithelial-mesenchymal-transition-in-pancreatic-cancer-cells-via-tgf-%C3%AE-smad-signalling
#18
Min Xu, Junbo He, Jie Li, Wen Feng, Hailang Zhou, Hong Wei, Meng Zhou, Ying Lu, Jian Zeng, Wanxin Peng, Fengyi Du, Aihua Gong
BACKGROUND: Methyl-CpG-binding domain 3 (MBD3) is an aberrant expression in human malignancies. However, the role of MBD3 in pancreatic cancer progression remains to be clarified. In this study, we investigated the effects of MBD3 on the epithelial-mesenchymal transition (EMT), and the underlying mechanism in pancreatic cancer cells. METHODS: The abilities of migration and invasion were examined by transwell and BD Matrigel invasion assays. EMT and TGF-β/Smad signalling were evaluated...
November 29, 2016: British Journal of Cancer
https://www.readbyqxmd.com/read/27896518/analysis-of-predictors-of-resection-and-survival-in-locally-advanced-stage-iii-pancreatic-cancer-does-the-nature-of-chemotherapy-regimen-influence-outcomes
#19
Filip Bednar, Mazen S Zenati, Jennifer Steve, Sharon Winters, Lee M Ocuin, Nathan Bahary, Melissa E Hogg, Herbert J Zeh, Amer H Zureikat
BACKGROUND: Locally advanced unresectable pancreatic cancer (LAPC) historically portends a poor prognosis. FOLFIRINOX and gemcitabine/nab-paclitaxel have proven effective in the metastatic setting. We sought to evaluate the outcomes of these regimens compared with older regimens in LAPC. METHODS: A retrospective, single institutional review of all consecutive LAPC treated with "new" (FOLFIRINOX and/or gemcitabine/nab-paclitaxel) and "old" (gemcitabine or 5-FU) chemotherapy from 2010 to 2014 was performed...
November 28, 2016: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/27895771/co-culture-of-hepatoma-cells-with-hepatocytic-precursor-stem-like-cells-inhibits-tumor-cell-growth-and-invasion-by-downregulating-akt-nf-%C3%AE%C2%BAb-expression
#20
Cheng-Jun Sui, Miao Xu, Wei-Qing Li, Jia-Mei Yang, Hong-Zhu Yan, Hui-Min Liu, Chun-Yan Xia, Hong-Yu Yu
Hepatocytic stem cells (HSCs) have inhibitory effects on hepatocarcinoma cells. The present study investigated the effects of HSC activity in hepatocarcinoma cells in vitro. A Transwell co-culture system of hepatocytic precursor (stem-like) WB-F344 cells and hepatoma CBRH-7919 cells was used to assess HSC activity in metastasized hepatoma cells in vitro. Nude mouse xenografts were used to assess HSC activity in vivo. Co-culture of hepatoma CBRH-7919 cells with WB-F344 cells suppressed the growth and colony formation, tumor cell migration and invasion capacity of CBRH-7919 cells...
November 2016: Oncology Letters
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