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Stem cell pulmonary fibrosis

Jiurong Liang, Yanli Zhang, Ting Xie, Ningshan Liu, Huaiyong Chen, Yan Geng, Adrianne Kurkciyan, Jessica Monterrosa Mena, Barry R Stripp, Dianhua Jiang, Paul W Noble
Successful recovery from lung injury requires the repair and regeneration of alveolar epithelial cells to restore the integrity of gas-exchanging regions within the lung and preserve organ function. Improper regeneration of the alveolar epithelium is often associated with severe pulmonary fibrosis, the latter of which involves the recruitment and activation of fibroblasts, as well as matrix accumulation. Type 2 alveolar epithelial cells (AEC2s) are stem cells in the adult lung that contribute to the lung repair process...
October 3, 2016: Nature Medicine
Sophie Wecht, Mauricio Rojas
Mesenchymal stem cells (MSCs) are a populace of non-haematopoietic multipotent stromal cells, which have the ability to differentiate into tissue derived from a single germ layer. MSCs have been isolated from various sites, including adipose tissue, skeletal muscle, synovium, spleen, thymus, lung and amniotic fluid, but are most often isolated from bone marrow. MSCs have several valuable functions that make them a promising therapeutic option in the field of regenerative medicine, including the secretion of anti-inflammatory cytokines and growth factors, the migration of cells to the site of injury when administered and the ability to 'rescue' cells through the transfer of functional mitochondria...
November 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
Jin Tao, Qinglun Li, Xiaowei Ma, Fei Han, Xiaoming Liu, Jun Wei, Yongzhao Zhu
Objective To investigate the therapeutic effect and mechanism of human placental mesenchymal stem cells of fetal origin (hfPMSCs) cultured in serum-free medium on mouse pulmonary fibrosis induced by bleomycin treatment. Methods Human hfPMSCs were cultured and identified by flow cytometry. Fifteen 6-week-old male SPF C57BL/6J mice were divided into 3 groups: bleomycin treatment group, hfPMSCs transplantation group and negative control group. Pulmonary fibrosis model was induced in the mice of bleomycin treatment group and hfPMSCs transplantation group with bleomycin (1 μg/L, 50 μL) via intratracheal instillation...
October 2016: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
Wook-Jin Chung
Pulmonary arterial hypertension (PAH) is a life-threatening disorder with a poor prognosis and causes pulmonary vascular remodeling accompanied with increased pulmonary arterial medial wall thickness and fibrosis, which leads to vascular and right ventricular (RV) dysfunction. Despite treatment with prostacyclin, endothelin antagonist, and phosphodiesterase-5 inhibitors the 1-year mortality rate of PAH still remains high. Recent registries, clinical trials, and basic researches have been increasing the knowledge of PAH and it would contribute to potential therapeutic strategies and better clinical outcome...
September 2016: Journal of Hypertension
Dan C Wilkinson, Jackelyn A Alva-Ornelas, Jennifer M S Sucre, Preethi Vijayaraj, Abdo Durra, Wade Richardson, Steven J Jonas, Manash K Paul, Saravanan Karumbayaram, Bruce Dunn, Brigitte N Gomperts
: : Stem cell technologies, especially patient-specific, induced stem cell pluripotency and directed differentiation, hold great promise for changing the landscape of medical therapies. Proper exploitation of these methods may lead to personalized organ transplants, but to regenerate organs, it is necessary to develop methods for assembling differentiated cells into functional, organ-level tissues. The generation of three-dimensional human tissue models also holds potential for medical advances in disease modeling, as full organ functionality may not be necessary to recapitulate disease pathophysiology...
September 15, 2016: Stem Cells Translational Medicine
Shaimaa Elmahadi, Hideki Muramatsu, Seiji Kojima
PURPOSE OF REVIEW: Dyskeratosis congenita is an inherited bone marrow failure syndrome caused by defects in telomere maintenance. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for bone marrow failure because of dyskeratosis congenita. The present review summarizes the literature with respect to the diagnosis and treatment of patients with dyskeratosis congenita who received HSCT, and discusses the recent progress in the management of dyskeratosis congenita...
November 2016: Current Opinion in Hematology
S X Yang, Q Wu, X Sun, X Li, K Li, L Xu, Y Li, Q Y Zhang, Y C Zhang, H Y Chen
OBJECTIVE: To investigate the effect of fibroblasts on regulating airway stem cell proliferation in idiopathic pulmonary fibrosis. METHODS: Lung cell suspension was prepared from β-actin-GFP mice. Airway stem cells were obtained by fluorescence activated cell sorting and co-cultured with lung fibroblasts. The fibroblasts were treated with TGF-β inhibitor SB43142. The expression of growth factors FGF1/2 and the effect of FGF1/2 on stem cell proliferation were observed...
September 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
Rebecca L Heise, Patrick A Link, Laszlo Farkas
The field of stem cell biology, cell therapy, and regenerative medicine has expanded almost exponentially, in the last decade. Clinical trials are evaluating the potential therapeutic use of stem cells in many adult and pediatric lung diseases with vascular component, such as bronchopulmonary dysplasia (BPD), chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), or pulmonary arterial hypertension (PAH). Extensive research activity is exploring the lung resident and circulating progenitor cells and their contribution to vascular complications of chronic lung diseases, and researchers hope to use resident or circulating stem/progenitor cells to treat chronic lung diseases and their vascular complications...
2016: Frontiers in Pediatrics
Simon A Filson, Aviad Keren, Nyra Goldstein, Yehuda Ullmann
BACKGROUND: Fat grafting is an increasingly popular method of augmentation/reconstruction of soft tissue defects. However, the clinical unpredictability and high resorption rates of the grafts remain problematic. Cellular stress from the harvest and the ensuing ischemic episode may be the cause of this. Cellular stress activates the p38 mitogen-activated protein kinase (MAPK) signaling pathway. In response to cellular stress, the p38 pathway can lead to apoptosis and can negatively regulate cell proliferation...
July 2016: Plastic and Reconstructive Surgery. Global Open
Darcy E Wagner, Wellington V Cardoso, Sarah E Gilpin, Susan Majka, Harald Ott, Scott H Randell, Bernard Thébaud, Thomas Waddell, Daniel J Weiss
The University of Vermont College of Medicine, in collaboration with the NHLBI, Alpha-1 Foundation, American Thoracic Society, Cystic Fibrosis Foundation, European Respiratory Society, International Society for Cellular Therapy, and the Pulmonary Fibrosis Foundation, convened a workshop, "Stem Cells and Cell Therapies in Lung Biology and Lung Diseases," held July 27 to 30, 2015, at the University of Vermont. The conference objectives were to review the current understanding of the role of stem and progenitor cells in lung repair after injury and to review the current status of cell therapy and ex vivo bioengineering approaches for lung diseases...
August 2016: Annals of the American Thoracic Society
Anandharajan Rathinasabapathy, Erin Bruce, Andrew Espejo, Alana Horowitz, Dhivya R Sudhan, Anand Nair, Dominic Guzzo, Joseph Francis, Mohan K Raizada, Vinayak Shenoy, Michael J Katovich
BACKGROUND AND PURPOSE: Pulmonary hypertension (PH) and pulmonary fibrosis (PF) are life threatening cardiopulmonary diseases. Existing pharmacological interventions have failed to improve clinical outcomes or reduce disease-associated mortality. Emerging evidence suggests that stem cells offer an effective treatment approach against various pathological conditions. It has been proposed that their beneficial actions may be mediated via secretion of paracrine factors. Herein, we evaluated the therapeutic potential of conditioned media (CM) from adipose stem cells (ASCs) against experimental models of PH and PF...
October 2016: British Journal of Pharmacology
Cong Wang, Shen Gu, Honghui Cao, Zutong Li, Zou Xiang, Kebin Hu, Xiaodong Han
Myofibroblast differentiation of lung resident mesenchymal stem cells (LR-MSC) plays an important role in idiopathic pulmonary fibrosis. By comparing the expression profiles of miRNAs before and after myofibroblast differentiation of LR-MSC, we identified miR-877-3p as a fibrosis-related miRNA. We found that miR-877-3p sequestration inhibited the myofibroblast differentiation of LR-MSC and attenuates bleomycin-induced lung fibrosis by targeting Smad7. Smad7, as an inhibitory smad in the TGF-β1 signaling pathway, was decreased in the myofibroblast differentiation of LR-MSC and up-regulation of Smad7 could inhibit the differentiation process...
2016: Scientific Reports
Øyvind Molberg, Anna-Maria Hoffmann-Vold
PURPOSE OF REVIEW: Interstitial lung disease (ILD) is the major determinant of morbidity and mortality in systemic sclerosis (SSc). In highly selected SSc patients, it was recently shown that stem cell therapy early in the disease course improved survival and reduced the extent of ILD, providing a rationale for early ILD detection strategies in this disease. Here, we review recent progress on ILD screening and early diagnosis in SSc. RECENT FINDINGS: Two studies showed that over 60% of unselected SSc cases with ILD by high-resolution computer tomography (HRCT) had normal range pulmonary function tests (PFTs); indicating poor performance of PFTs for ILD screening purposes...
November 2016: Current Opinion in Rheumatology
Jean Ann Maguire, Lin Lu, Jason A Mills, Lisa M Sullivan, Paul Gadue, Deborah L French
Hermansky-Pudlak syndrome type 2 (HPS2) is a rare autosomal recessive disorder resulting from functional mutations in the adaptor-related protein complex 3, beta 1 subunit (AP3B1) gene. This gene plays a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. Here we describe the generation of an HPS2 iPS cell line (CHOPHPS2) using a Cre-excisable polycistronic STEMCCA lentivirus. This line was derived from human fibroblasts isolated from a patient carrying two mutations in the AP3B1 gene...
March 2016: Stem Cell Research
Jean Ann Maguire, Lin Lu, Jason A Mills, Lisa M Sullivan, Alyssa Gagne, Paul Gadue, Deborah L French
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by deficiencies in lysosome-related organelles such as melanosomes and platelet-dense granules. The disorder is classified into nine different subtypes (HPS1-HPS9) based on genetic mutations in 9 unique genes. Here we describe the generation of an HPS1 iPSC line (CHOPHPS1) using a Cre-excisable polycistronic STEMCCA lentivirus. This line was derived from human fibroblasts isolated from a patient carrying a duplicative mutation in the HPS1 gene...
March 2016: Stem Cell Research
Christopher Kloth, Wolfgang Maximilian Thaiss, Heike Preibsch, Klemens Mark, Ina Kötter, Jürgen Hetzel, Konstantin Nikolaou, Jörg Henes, Marius Horger
OBJECTIVES: The aim of this study was to evaluate the course of SSc-related pulmonary abnormalities following high-dose chemotherapy with autologous stem cell transplantation (SCT) by quantitative chest CT analysis and compare the results with those of pulmonary function tests and the response of cutaneous involvement. METHODS: Chest CT quantification was performed before, directly after [0.49 years (sd 0.20)] and at a mean of 2.2 years (sd 2.1) following autologous SCT in 26 consecutive patients with SSc between March 2001 and March 2015...
October 2016: Rheumatology
Takahiro Matsui, Tetsuo Maeda, Toru Kida, Jiro Fujita, Hiromi Tsuji, Eiichi Morii, Yuzuru Kanakura
Late-onset noninfectious pulmonary complication after allogenic hematopoietic stem cell transplantation is an important contributing factor associated with high rate morbidity and mortality. We report a case with pleuroparenchymal fibroelastosis (PPFE) occurred after allogenic bone marrow transplantation. The onset was infiltrative shadows in upper lobes, and the haziness spread gradually throughout the lungs with recurrent episodes of pneumothorax in both lungs. Progressive respiratory failure in course of adrenocortical steroid administration eventually caused death...
October 2016: International Journal of Hematology
Sara Piciucchi, Sara Tomassetti, Claudia Ravaglia, Christian Gurioli, Carlo Gurioli, Alessandra Dubini, Angelo Carloni, Marco Chilosi, Thomas V Colby, Venerino Poletti
BACKGROUND: The diagnostic and prognostic impact of traction bronchiectasis on high resolution CT scan (HRCT) in patients suspected to have idiopathic pulmonary fibrosis (IPF) is increasing significantly. MAIN BODY: Recent data demonstrated that cysts in honeycombing areas are covered by epithelium expressing bronchiolar markers. In IPF bronchiolization is the final consequence of a variety of pathogenic events starting from alveolar stem cell exhaustion, and ending in a abnormal/dysplastic proliferation of bronchiolar epithelium...
2016: BMC Pulmonary Medicine
Barry Weinberger, Rama Malaviya, Vasanthi R Sunil, Alessandro Venosa, Diane E Heck, Jeffrey D Laskin, Debra L Laskin
Most mortality and morbidity following exposure to vesicants such as sulfur mustard is due to pulmonary toxicity. Acute injury is characterized by epithelial detachment and necrosis in the pharynx, trachea and bronchioles, while long-term consequences include fibrosis and, in some instances, cancer. Current therapies to treat mustard poisoning are primarily palliative and do not target underlying pathophysiologic mechanisms. New knowledge about vesicant-induced pulmonary disease pathogenesis has led to the identification of potentially efficacious strategies to reduce injury by targeting inflammatory cells and mediators including reactive oxygen and nitrogen species, proteases and proinflammatory/cytotoxic cytokines...
August 15, 2016: Toxicology and Applied Pharmacology
Udit Agarwal, Amanda W Smith, Kristin M French, Archana V Boopathy, Alex George, David Trac, Milton E Brown, Ming Shen, Rong Jiang, Janet D Fernandez, Brian E Kogon, Kirk R Kanter, Baahaldin Alsoufi, Mary B Wagner, Manu O Platt, Michael E Davis
UNLABELLED: Children with congenital heart diseases have increased morbidity and mortality, despite various surgical treatments, therefore warranting better treatment strategies. Here we investigate the role of age of human pediatric cardiac progenitor cells (hCPCs) on ventricular remodeling in a model of juvenile heart failure. hCPCs isolated from children undergoing reconstructive surgeries were divided into 3 groups based on age: neonate (1 day to 1 month), infant (1 month to 1 year), and child (1 to 5 years)...
July 2016: Stem Cells Translational Medicine
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