keyword
MENU ▼
Read by QxMD icon Read
search

SMA mice

keyword
https://www.readbyqxmd.com/read/28426667/bioenergetic-status-modulates-motor-neuron-vulnerability-and-pathogenesis-in-a-zebrafish-model-of-spinal-muscular-atrophy
#1
Penelope J Boyd, Wen-Yo Tu, Hannah K Shorrock, Ewout J N Groen, Roderick N Carter, Rachael A Powis, Sophie R Thomson, Derek Thomson, Laura C Graham, Anna A L Motyl, Thomas M Wishart, J Robin Highley, Nicholas M Morton, Thomas Becker, Catherina G Becker, Paul R Heath, Thomas H Gillingwater
Degeneration and loss of lower motor neurons is the major pathological hallmark of spinal muscular atrophy (SMA), resulting from low levels of ubiquitously-expressed survival motor neuron (SMN) protein. One remarkable, yet unresolved, feature of SMA is that not all motor neurons are equally affected, with some populations displaying a robust resistance to the disease. Here, we demonstrate that selective vulnerability of distinct motor neuron pools arises from fundamental modifications to their basal molecular profiles...
April 20, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28412345/a-novel-identified-circular-rna-circrna_010567-promotes-myocardial-fibrosis-via-suppressing-mir-141-by-targeting-tgf-%C3%AE-1
#2
Bing Zhou, He-Yun Xu
Circular RNAs (circRNAs) are a novel type of endogenous noncoding RNA gaining research interest in recent years. Despite this increase in interest, the mechanism of circRNAs in the pathogenesis of multiple cardiovascular diseases, particularly myocardial fibrosis, is rarely reported. In the following study, the expression profiles and potential mechanisms of circRNAs in mice myocardial fibrosis models in vitro are investigated. Previous research examining circRNA expression profiles of diabetic db/db mice myocardium using circRNA microarray found 43 circRNAs were abnormally expressed, including 24 up-regulated circRNAs and 19 down-regulated circRNAs...
April 12, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28412171/gender-specific-amelioration-of-sma-phenotype-upon-disruption-of-a-deep-intronic-structure-by-an-oligonucleotide
#3
Matthew D Howell, Eric W Ottesen, Natalia N Singh, Rachel L Anderson, Ravindra N Singh
Spinal muscular atrophy (SMA), the leading genetic disease of children, is caused by low levels of survival motor neuron (SMN) protein. Here, we employ A15/283, an antisense oligonucleotide targeting a deep intronic sequence/structure, to examine the impact of restoration of SMN in a mild SMA mouse model. We show gender-specific amelioration of tail necrosis upon subcutaneous administrations of A15/283 into SMA mice at postnatal days 1 and 3. We also demonstrate that a modest increase in SMN due to early administrations of A15/283 dramatically improves testicular development and spermatogenesis...
April 13, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28409388/diethylcarbamazine-attenuates-the-expression-of-pro-fibrogenic-markers-and-hepatic-stellate-cells-activation-in-carbon-tetrachloride-induced-liver-fibrosis
#4
Maria Eduarda Rocha de França, Sura Wanessa Santos Rocha, Wilma Helena Oliveira, Laise Aline Santos, Anne Gabrielle Vasconcelos de Oliveira, Karla Patrícia Sousa Barbosa, Ana Karolina Santana Nunes, Gabriel Barros Rodrigues, Deniele Bezerra Lós, Christina Alves Peixoto
BACKGROUND AND AIM: While diethylcarbamazine citrate (DEC) displays important anti-inflammatory effects in experimental models of liver injury, the mechanisms of its action remain poorly understood. The aim of the present study was to investigate the fibrolytic potential of DEC. METHODS: Mice receive two injections of carbon tetrachloride (CCl4) per week for 8 weeks. DEC 50 mg/kg body weight was administered through drinking water during the last 12 days of liver injury...
April 13, 2017: Inflammopharmacology
https://www.readbyqxmd.com/read/28408888/prevention-of-bleomycin-induced-pulmonary-inflammation-and-fibrosis-in-mice-by-paeonol
#5
Meng-Han Liu, An-Hsuan Lin, Hsin-Kuo Ko, Diahn-Warng Perng, Tzong-Shyuan Lee, Yu Ru Kou
Pulmonary fibrosis is a severe and progressive disease that is characterized by an abnormal deposition of extracellular matrix, such as collagens. The pathogenesis of this disease may be initiated by oxidative damage of lung epithelial cells by fibrogenic stimuli, leading to lung inflammation, which in turn promotes various lung fibrotic responses. The profibrogenic effect of transforming growth factor-β1 (TGF-β1) on lung fibroblasts is crucial for the pathogenesis of this disease. Paeonol, the main phenolic compound present in the Chinese herb Paeonia suffruticosa, has antioxidant and anti-inflammatory properties...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28408805/effect-of-berberine-on-the-renal-tubular-epithelial-to-mesenchymal-transition-by-inhibition-of-the-notch-snail-pathway-in-diabetic-nephropathy-model-kkay-mice
#6
Guannan Yang, Zongjiang Zhao, Xinxue Zhang, Amin Wu, Yawei Huang, Yonghui Miao, Meijuan Yang
Renal tubular epithelial-to-mesenchymal transition (EMT) and renal tubular interstitial fibrosis are the main pathological changes of diabetic nephropathy (DN), which is a common cause of end-stage renal disease. Previous studies have suggested that berberine (BBR) has antifibrotic effects in the kidney and can reduce apoptosis and inhibit the EMT of podocytes in DN. However, the effect of BBR on the renal tubular EMT in DN and its mechanisms of action are unknown. This study was performed to explore the effects of BBR on the renal tubular EMT and the molecular mechanisms of BBR in DN model KKAy mice and on the high glucose (HG)-induced EMT in mouse renal tubular epithelial cells...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28393518/enhancing-vascularization-through-the-controlled-release-of-platelet-derived-growth-factor-bb
#7
Silvia Minardi, Laura Pandolfi, Francesca Taraballi, Xin Wang, Enrica De Rosa, Zachary D Mills, Xuewu Liu, Mauro Ferrari, Ennio Tasciotti
Using delivery systems to control the in vivo release of growth factors (GFs) for tissue engineering applications is extremely desirable as the clinical use of GFs is limited by their fast in vivo turnover. Hence, the development of effective platforms that are able to finely control the release of GFs in vivo remains a challenge. Herein, we investigated the ability of multiscale microspheres, composed by a nanostructured silicon multistage vector (MSV) core and a poly(dl-lactide-co-glycolide) acid (PLGA) forming outer shell (PLGA-MSV), to release functional platelet-derived growth factor-BB (PDGF-BB) to induce in vivo localized neovascularization...
April 18, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28390311/metformin-mitigates-carbon-tetrachloride-induced-tgf-%C3%AE-1-smad3-signaling-and-liver-fibrosis-in-mice
#8
Kerui Fan, Kejia Wu, Ling Lin, Pu Ge, Jie Dai, Xiaojiao He, Kai Hu, Li Zhang
Increasing evidence suggests that the widely used antidiabetic drug metformin have an extensive therapeutic range beyond hypoglycemic therapy. We previously found that metformin significantly attenuated acute hepatitis. The present study investigated the potential effects of metformin on carbon tetrachloride (CCl4)-induced liver fibrosis in mice. The results indicated that treatment with metformin suppressed CCl4-induced elevation of liver index, formation of fibrotic septa, accumulation of connective tissue and upregulation of collagen I...
April 5, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28386330/geniposide-inhibited-endothelial-mesenchymal-transition-via-the-mtor-signaling-pathway-in-a-bleomycin-induced-scleroderma-mouse-model
#9
Qing Qi, Yueping Mao, Yongzhen Tian, Ke Zhu, Xushan Cha, Minghua Wu, Xiaodong Zhou
AIM: Geniposide is an iridoid glycoside isolated from the gardenia plant. It has multiple biological activities. The roles of geniposide in systemic sclerosis (SSc) and in endothelial-to-mesenchymal transition (EndMT) are unclear. We investigated the protective effects of geniposide in a bleomycin-induced SSc mouse model, and its potential mechanisms. METHODS: The effects of geniposide were evaluated as follows: (1) histological and immunochemical changes in mouse skin tissue; (2) changes in cellular morphology of human umbilical vein endothelial cells (HUVECs); (3) expression of endothelial cell biomarkers (E-Cadherin, CD31, and CD34), mesenchymal cell markers (FSP1, Collagen, and α-SMA), and key factors of EndMT (Slug, Snail, and Twist) using real time PCR, Western blot, and immunofluorescence; (4) tube formation in HUVECs; (5) mTOR signaling pathway transcription factors using Western blot analysis...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28385812/sirtuin-7-is-decreased-in-pulmonary-fibrosis-and-regulates-the-fibrotic-phenotype-of-lung-fibroblasts
#10
Anne Elizabeth Wyman, Zahid Noor, Rita Fishelevich, Virginia Lockatell, Nirav G Shah, Nevins W Todd, Sergei P Atamas
Pulmonary fibrosis is a severe condition with no cure and limited therapeutic options. Better understanding of its pathophysiology is needed. Recent studies have suggested that pulmonary fibrosis may be driven by accelerated aging-related mechanisms. Sirtuins (SIRTs), particularly SIRT1, -3, and -6, are well-known mediators of aging, however limited data exist on the contribution of sirtuins to lung fibrosis. We assessed the mRNA and protein levels of all seven known sirtuins in primary lung fibroblasts from patients with idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in comparison with lung fibroblasts from healthy controls...
April 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28384213/prevention-of-tgf-%C3%AE-induced-early-liver-fibrosis-by-a-maleic-acid-derivative-anti-oxidant-through-suppression-of-ros-inflammation-and-hepatic-stellate-cells-activation
#11
Kun-Lin Yang, Wen-Teng Chang, Ming-Yuan Hong, Kuo-Chen Hung, Chia-Chang Chuang
Current anti-fibrotic effect of antioxidants in vivo is disappointing due probably to the fact that once liver fibrogenesis is established it is too advanced to be reversed by anti-oxidation mechanism. We consider antioxidant may only act on the early phase of fibrogenesis. Thus, we had previously established an early liver fibrosis animal model using an inducible expression vector (pPK9a), which contains TGF-β gene and was hydro-dynamically transferred into mice to induce a transient liver fibrosis. TGF-β1 has been well documented to up-regulate the expression of α2(1) collagen (Col 1A2) gene in the liver via the reactive oxygen species (ROS); the process triggers inflammation, leading to hepatic stellate cells (HSC) activation and liver fibrogenesis...
2017: PloS One
https://www.readbyqxmd.com/read/28381600/increased-neutrophil-gelatinase-associated-lipocalin-ngal-promotes-airway-remodelling-in-chronic-obstructive-pulmonary-disease
#12
Yujie Wang, Man Jia, Xiaoyi Yan, Limin Cao, Peter J Barnes, Ian M Adcock, Mao Huang, Xin Yao
Airway remodelling is an important component of chronic obstructive pulmonary disease (COPD). Neutrophil gelatinase-associated lipocalin (NGAL) from neutrophils may drive COPD epithelial-mesenchymal transition (EMT). NGAL expression was quantified in the lungs of COPD patients and bronchoalveolar lavage fluid (BALF) of ozone-treated mice. Reticular basement membrane (RBM) thickness and E-cadherin and α-smooth muscle actin (α-SMA) expression were determined in mice airways. Effects of cigarette smoke extract (CSE) and inflammatory factors on NGAL expression in human neutrophils as well as the effects of NGAL on airway structural cells was assessed...
April 5, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28379958/il-15-regulates-migration-invasion-angiogenesis-and-genes-associated-with-lipid-metabolism-and-inflammation-in-prostate-cancer
#13
Krizia Rohena-Rivera, María M Sánchez-Vázquez, Diana A Aponte-Colón, Ingrid S Forestier-Román, Mario E Quintero-Aguiló, Magaly Martínez-Ferrer
Prostate cancer (PCa) is the most commonly diagnosed non-cutaneous cancer. In the United States it is second leading cause of cancer related deaths in men. PCa is often treated via radical prostatectomy (RP). However, 15-30% of the patients develop biochemical recurrence (i.e. increased serum prostate specific antigen (PSA) levels). Interleukin-15 (IL-15) is a secreted cytokine found over expressed in patients with recurrence-free survival after RP. In our study, we aim to determine the role of IL-15 in PCa using in vitro and in vivo models, and gene expression analysis...
2017: PloS One
https://www.readbyqxmd.com/read/28379354/motor-neuronal-repletion-of-the-nmj-organizer-agrin-modulates-the-severity-of-the-spinal-muscular-atrophy-disease-phenotype-in-model-mice
#14
Jeong-Ki Kim, Charlotte Caine, Tomoyuki Awano, Ruth Herbst, Umrao R Monani
Spinal muscular atrophy (SMA) is a common and often fatal neuromuscular disorder caused by low levels of the Survival Motor Neuron (SMN) protein. Amongst the earliest detectable consequences of SMN deficiency are profound defects of the neuromuscular junctions (NMJs). In model mice these synapses appear disorganized, fail to mature and are characterized by poorly arborized nerve terminals. Given one role of the SMN protein in orchestrating the assembly of spliceosomal snRNP particles and subsequently regulating the alternative splicing of pre-mRNAs, a plausible link between SMN function and the distal neuromuscular SMA phenotype is an incorrectly spliced transcript or transcripts involved in establishing or maintaining NMJ structure...
March 31, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28364627/protective-role-of-rhapontin-in-experimental-pulmonary-fibrosis-in-vitro-and-in-vivo
#15
Lijun Tao, Juan Cao, Wencheng Wei, Haifeng Xie, Mian Zhang, Chaofeng Zhang
BACKGROUND: Pulmonary fibrosis is a scaring process related to chronic lung injury of all causes. The treatment options for pulmonary fibrosis are very limited. Rhapontin has anti-inflammatory effect and anti-proliferative activity which is widely distributed in the medicinal plants of Rheum genus in China. However, the anti-fibrotic activities of rhapontin have not been previously investigated. METHODS: The effect of rhapontin on TGF-β1-mediated extracellular matrix (ECM) deposition in primary lung fibroblast (PLF) cells, on TGF-β1 secretion in LPS-stimulated human THP-1 derived macrophages in vitro, and on bleomycin (BLM)-induced pulmonary fibrosis was investigated in vivo...
March 29, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28364247/intracerebroventricular-delivery-in-mice-for-motor-neuron-diseases
#16
M Nizzardo, M Rizzuti
The use of antisense oligonucleotides to target specific mRNA sequences represents a promising therapeutic strategy for neurological disorders. Recent advances in antisense technology enclose the development of phosphorodiamidate morpholino oligomers (MO), which is one of the best candidates for molecular therapies due to MO's excellent pharmacological profile.Nevertheless, the route of administration of antisense compounds represents a critical issue in the neurological field. Particularly, as regards motor neuron diseases, intracerebroventricular (ICV) injection is undoubtedly the most efficient procedure to directly deliver therapeutic molecules in the central nervous system (CNS)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28364097/-effect-of-lamivudine-and-silymarin-on-liver-fibrosis-relevant-factors-in-hbv-transgenic-mice-with-alcohol-drinking
#17
Juanjuan Huang, Shikun Liu, Zuojun Li, Libo Cao, Linqi Ouyang
To observe the role of lamividine and silymarin preventing and curing liver fibrosis-relevant factors induced by alcohol drinking in hepatitis B virus (HBV) transgenic mice (Tg mice).
 Methods: Forty HBV-Tg BALB/C mice with 1.3 copy were randomly divided into 4 groups: a control group, a model group, a lamivudine group and a silymarin group. Tg mice in control group were treated with normal saline via intragastric administration; Tg-mice in the model group were treated with 50% alcohol (5 mL/kg) once a day via intragastric administration; while Tg-mice in lamivudine group and silymarin group were treated with alcohol (5 mL/kg) plus laminvudine (100 mg/kg) and silymarin (200 mg/kg) once a day via intragastric administration respectively...
March 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28362802/comparison-of-independent-screens-on-differentially-vulnerable-motor-neurons-reveals-alpha-synuclein-as-a-common-modifier-in-motor-neuron-diseases
#18
Rachel A Kline, Kevin A Kaifer, Erkan Y Osman, Francesco Carella, Ariana Tiberi, Jolill Ross, Giuseppa Pennetta, Christian L Lorson, Lyndsay M Murray
The term "motor neuron disease" encompasses a spectrum of disorders in which motor neurons are the primary pathological target. However, in both patients and animal models of these diseases, not all motor neurons are equally vulnerable, in that while some motor neurons are lost very early in disease, others remain comparatively intact, even at late stages. This creates a valuable system to investigate the factors that regulate motor neuron vulnerability. In this study, we aim to use this experimental paradigm to identify potential transcriptional modifiers...
March 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28355745/-cinnamaldehyde-attenuates-pressure-overload-induced-cardiac-fibrosis-via-inhibition-of-endothelial-mesenchymal-transition
#19
Y Xiao, Q Q Wu, X H Jiang, Q Z Tang
Objective: The aim of this study is to investigate the underlying mechanism of cinnamaldehyde attenuating pressure overload-induced cardiac fibrosis. Methods: The mice were randomly divided into control group, model group and treatment group by random number table and each group had 8 mice.Cardiac hypertrophy was induced by aortic banding. Heart vascular density was detected by immunohistochemical staining of CD31.The expression level of stromal cells marker α-smooth muscle actin (α-SMA) was detected by immunofluorescence staining in different groups...
March 21, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28350070/tieg1-deficiency-confers-enhanced-myocardial-protection-in-the-infarcted-heart-by-mediating-the-pten-akt-signalling-pathway
#20
Mingqiu Cen, Pengfei Hu, Zhaobin Cai, Tianfu Fang, Jiancheng Zhang, Ming Lu
The transforming growth factor (TGF)-β-inducible early gene-1 (TIEG1) plays a crucial role in modulating cell apoptosis and proliferation in a number of diseases, including pancreatic cancer, leukaemia and osteoporosis. However, the functional role of TIEG1 in the heart has not been fully defined. In this study, we first investigated the role of TIEG1 in ischaemic heart disease. For in vitro experiments, cardiomyocytes were isolated from both TIEG1 knockout (KO) and wile-type (WT) mice, and the apoptotic ratios were evaluated after a 48‑h ischaemic insult...
February 13, 2017: International Journal of Molecular Medicine
keyword
keyword
70362
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"