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https://www.readbyqxmd.com/read/29453127/generation-of-d1-1-talen-isogenic-control-cell-line-from-dravet-syndrome-patient-ipscs-using-talen-mediated-editing-of-the-scn1a-gene
#1
Yasuyoshi Tanaka, Takefumi Sone, Norimichi Higurashi, Tetsushi Sakuma, Sadafumi Suzuki, Mitsuru Ishikawa, Takashi Yamamoto, Jun Mitsui, Hitomi Tsuji, Hideyuki Okano, Shinichi Hirose
Dravet syndrome (DS) is an infantile epileptic encephalopathy mainly caused by de novo mutations in the SCN1A gene encoding the α1 subunit of the voltage-gated sodium channel Na v 1.1. As an in vitro model of this disease, we previously generated an induced pluripotent stem cell (iPSC) line from a patient with DS carrying a c.4933C>T (p.R1645*) substitution in SCN1A. Here, we describe developing a genome-edited control cell line from this DS iPSC line by substituting the point mutation with the wild-type residue...
February 2, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29452534/co-culture-of-human-bone-marrow-mesenchymal-stem-cells-and-macrophages-attenuates-lipopolysaccharide-induced-inflammation-in-human-corneal-epithelial-cells
#2
Won-Yong Jeong, Ji-Hye Kim, Chan-Wha Kim
Dry eye syndrome (DES) is considered as an ocular surface inflammatory disease. Previous studies have shown inflammation plays an important role in the progression and onset of DES. Co-culture of human bone marrow mesenchymal stem cells (HBMSCs) and macrophages showed immunomodulatory effects via regulation of cytokine regulation. Thus, the aim of this study was to investigate the effect of the interaction of these cells on in vitro DES model. The conditioned media (CM) from macrophages, HBMSCs, and HBMSCs + macrophages were treated to human corneal epithelial cells, which showed significant reduction in IL-1α and IL-1β expression levels in HBMSCs + macrophages group...
February 16, 2018: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/29452453/-personalized-ophthalmology-induced-pluripotent-stem-cells-for-in-vitro-modelling-of-retinal-degenerative-diseases
#3
Caroline Brandl, Bernhard H F Weber
Today, the search for therapeutic options to treat retinal degeneration often relies on an in-depth understanding of the underlying pathological events. Alternatively, it is conceivable to search, in an undirected screening approach, for chemical compounds affecting disease outcome. For both approaches, there is an urgent need for in vitro and, ideally, in vivo disease models that adequately reflect the site of pathology. Currently available animal models possess limitations as they often develop only defined aspects of disease...
February 16, 2018: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/29452156/establishment-of-a-prkag2-cardiac-syndrome-disease-model-and-mechanism-study-using-human-induced-pluripotent-stem-cells
#4
Yongkun Zhan, Xiaolei Sun, Bin Li, Huanhuan Cai, Chen Xu, Qianqian Liang, Chao Lu, Ruizhe Qian, Sifeng Chen, Lianhua Yin, Wei Sheng, Guoying Huang, Aijun Sun, Junbo Ge, Ning Sun
PRKAG2 cardiac syndrome is a distinct form of human cardiomyopathy characterized by cardiac hypertrophy, ventricular pre-excitation and progressive cardiac conduction disorder. However, it remains unclear how mutations in the PRKAG2 gene give rise to such a complicated disease. To investigate the underlying molecular mechanisms, we generated disease-specific hiPSC-derived cardiomyocytes from two brothers both carrying a heterozygous missense mutation c.905G>A (R302Q) in the PRKAG2 gene and further corrected the R302Q mutation with CRISPR-Cas9 mediated genome editing...
February 13, 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/29451335/mesenchymal-stem-cells-protect-against-hypoxia-ischemia-brain-damage-by-enhancing-autophagy-through-bdnf-mtor-signaling-pathway
#5
Zhen Zheng, Li Zhang, Yi Qu, Guoguang Xiao, Shiping Li, Shan Bao, Richard Lu, Dezhi Mu
Hypoxic-ischemic encephalopathy (HIE) is a serious disease for neonates. However, present therapeutic strategies are not effective enough for treating HIE. Previous study showed that mesenchymal stem cells (MSCs) can exert neuroprotective effects for brain damage, but its mechanism remains elusive. Using in-vitro co-culture of rat cortical primary neurons and MSCs in HI conditions, we demonstrated that MSCs help increase BDNF and autophagy markers (LC3II and Beclin1) in the cultures and decrease cells death (LDH levels)...
February 16, 2018: Stem Cells
https://www.readbyqxmd.com/read/29449460/pfn2-and-gamt-as-common-molecular-determinants-of-axonal-charcot-marie-tooth-disease
#6
Manisha Juneja, Abdelkrim Azmi, Jonathan Baets, Andreas Roos, Matthew J Jennings, Paola Saveri, Chiara Pisciotta, Nathalie Bernard-Marissal, Bernard L Schneider, Catherine Verfaillie, Roman Chrast, Pavel Seeman, Angelika F Hahn, Peter de Jonghe, Stuart Maudsley, Rita Horvath, Davide Pareyson, Vincent Timmerman
BACKGROUND: Charcot-Marie-Tooth type 2 (CMT2) neuropathy is characterised by a vast clinical and genetic heterogeneity complicating its diagnosis and therapeutic intervention. Identification of molecular signatures that are common to multiple CMT2 subtypes can aid in developing therapeutic strategies and measuring disease outcomes. METHODS: A proteomics-based approach was performed on lymphoblasts from CMT2 patients genetically diagnosed with different gene mutations to identify differentially regulated proteins...
February 15, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29448133/exposure-to-the-rock-inhibitor-fasudil-promotes-gliogenesis-of-neural-stem-cells-in-vitro
#7
Zubair Ahmed Nizamudeen, Lisa Chakrabarti, Virginie Sottile
Fasudil is a clinically approved Rho-associated protein kinase (ROCK) inhibitor that has been used widely to treat cerebral consequences of subarachnoid hemorrhage. It is known to have a positive effect on animal models of neurological disorders including Parkinson's disease and stroke. However, its cellular effect on progenitor populations and differentiation is not clearly understood. While recent studies suggest that fasudil promotes the mobilization of neural stem cells (NSCs) from the subventricular zone in vivo and promotes the differentiation of the C17...
February 6, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29448001/induced-pluripotent-stem-cells-ipsc-derived-retinal-cells-in-disease-modeling-and-regenerative-medicine
#8
REVIEW
Reena Rathod, Harshini Surendran, Rajani Battu, Jogin Desai, Rajarshi Pal
Retinal degenerative disorders are a leading cause of the inherited, irreversible and incurable vision loss. While various rodent model systems have provided crucial information in this direction.lack of disease-relevant tissue availability and species-specific differences have proven to be a major roadblock. Human induced pluripotent stem cells (iPSC) have opened up a whole new avenue of possibilities not just in understanding the disease mechanism but also potential therapeutic approaches towards a cure. In this review, we have summarized recent advances in the methods of deriving retinal cell types from iPSCs which can serve as a renewable source of disease-relevant cell population for basic as well as translational studies...
February 12, 2018: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/29446747/crispr-cas9-genome-editing-a-promising-tool-for-therapeutic-applications-of-induced-pluripotent-stem-cells
#9
Yanli Zhang, Danuta Sastre, Feng Wang
Induced pluripotent stem cells hold tremendous potential for biological and therapeutic applications. The development of efficient technologies for the targeted genome alteration of stem cells in disease models is a prerequisite for utilizing stem cells to their full potential. The revolutionary technology for genome editing known as the clustered regularly interspaced short palindromic repeat (CRISPR)-associated protein 9 (Cas9) system is recently recognized as a powerful tool for editing DNA at specific loci...
February 14, 2018: Current Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29446074/a-hitchhiker-s-guide-to-humanized-mice-new-pathways-to-studying-viral-infections
#10
REVIEW
J K Skelton, A M Ortega-Prieto, M Dorner
Humanized mice are increasingly appreciated as an incredibly powerful platform for infectious disease research. The often very narrow species tropism of many viral infections, coupled with the sometimes-misleading results from preclinical studies in animal models (e.g. fatal immune reactions in the PEARL study 1 ) further emphasize the need for more predictive model systems based on human cells rather than surrogates. Humanized mice represent such a model and have been greatly enhanced in regards to their immune system reconstitution as well as immune functionality in the past years, resulting in their recommendation as pre-clinical model by the US Food and Drug Administration...
February 15, 2018: Immunology
https://www.readbyqxmd.com/read/29445623/bone-marrow-stem-cells-to-destroy-circulating-hiv-a-hypothetical-therapeutic-strategy
#11
Umesh Chandra Halder
Human immunodeficiency virus (HIV) still poses enigmatic threats to human life. This virus has mastered in bypassing anti retroviral therapy leading to patients' death. Circulating viruses are phenomenal for the disease outcome. This hypothesis proposes a therapeutic strategy utilizing receptor-integrated hematopoietic, erythroid and red blood cells. Here, HIV specific receptors trap circulating viruses that enter erythrocyte cytoplasm and form inactive integration complex. This model depicts easy, effective removal of circulating HIV without any adverse effect...
December 2018: Journal of Biological Research
https://www.readbyqxmd.com/read/29444913/lung-transplantation-after-allogeneic-stem-cell-transplantation-a-pan-european-experience
#12
Mark Greer, Cristina Berastegui, Peter Jaksch, Christian Benden, John Aubert, Antoine Roux, Elodie Lhuillier, Sandrine Hirschi, Martine Reynaud-Gaubert, François Philit, Johanna Claustre, Pierre LePalud, Marc Stern, Christiane Knoop, Robin Vos, Erik Verschuuren, Andrew Fisher, Gerdt Riise, Lennart Hansson, Martin Iversen, Pekka Hämmäinen, Hans Wedel, Jacqueline Smits, Jens Gottlieb, Are M Holm
Late-onset noninfectious pulmonary complications (LONIPCs) affect 6% of allogeneic stem cell transplantation (SCT) recipients within 5 years, conferring subsequent 5-year survival of 50%. Lung transplantation is rarely performed in this setting due to concomitant extrapulmonary morbidity, excessive immunosuppression and concerns about recurring malignancy being considered contraindications. This study assesses survival in highly selected patients undergoing lung transplantation for LONIPCs after SCT.SCT patients undergoing lung transplantation at 20 European centres between 1996 and 2014 were included...
February 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29444173/transcriptome-variations-among-human-embryonic-stem-cell-lines-are-associated-with-their-differentiation-propensity
#13
Changbin Sun, Jiawen Zhang, Dongmin Zheng, Jian Wang, Huanming Yang, Xi Zhang
Human embryonic stem cells (hESCs) have the potential to form any cell type in the body, making them attractive cell sources in drug screening, regenerative medicine, disease and developmental processes modeling. However, not all hESC lines have the equal potency to generate desired cell types in vitro. Significant variations have been observed for the differentiation efficiency of various human ESC lines. The precise underpinning molecular mechanisms are still unclear. In this work, we compared transcriptome variations of four hESC lines H7, HUES1, HUES8 and HUES9...
2018: PloS One
https://www.readbyqxmd.com/read/29443926/the-pleiotropic-effects-of-the-canonical-wnt-pathway-in-early-development-and-pluripotency
#14
REVIEW
Anchel de Jaime-Soguero, Willy Antoni Abreu de Oliveira, Frederic Lluis
The technology to derive embryonic and induced pluripotent stem cells from early embryonic stages and adult somatic cells, respectively, emerged as a powerful resource to enable the establishment of new in vitro models, which recapitulate early developmental processes and disease. Additionally, pluripotent stem cells (PSCs) represent an invaluable source of relevant differentiated cell types with immense potential for regenerative medicine and cell replacement therapies. Pluripotent stem cells support self-renewal, potency and proliferation for extensive periods of culture in vitro...
February 14, 2018: Genes
https://www.readbyqxmd.com/read/29443034/subretinal-transplantation-of-human-embryonic-stem-cell-derived-retinal-pigment-epithelial-cells-into-a-large-eyed-model-of-geographic-atrophy
#15
Sandra Petrus-Reurer, Hammurabi Bartuma, Monica Aronsson, Sofie Westman, Fredrik Lanner, Anders Kvanta
Geographic atrophy (GA), the late stage of dry age-related macular degeneration is characterized by loss of the retinal pigment epithelial (RPE) layer, which leads to subsequent degeneration of vital retinal structures (e.g., photoreceptors) causing severe vision impairment. Similarly, RPE-loss and decrease in visual acuity is seen in long-term follow up of patients with advanced wet age-related macular degeneration (AMD) receiving intravitreal anti-vascular endothelial growth factor (VEGF) treatment. Therefore, on the one hand, it is fundamental to efficiently derive RPE cells from an unlimited source that could serve as replacement therapy...
January 22, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29443032/kinetic-analysis-of-vasculogenesis-quantifies-dynamics-of-vasculogenesis-and-angiogenesis-in-vitro
#16
Kaela M Varberg, Seth Winfree, Kenneth W Dunn, Laura S Haneline
Vasculogenesis is a complex process by which endothelial stem and progenitor cells undergo de novo vessel formation. Quantitative assessment of vasculogenesis has become a central readout of endothelial progenitor cell functionality, and therefore, several attempts have been made to improve both in vitro and in vivo vasculogenesis models. However, standard methods are limited in scope, with static measurements failing to capture many aspects of this highly dynamic process. Therefore, the goal of developing this novel protocol was to assess the kinetics of in vitro vasculogenesis in order to quantitate rates of network formation and stabilization, as well as provide insight into potential mechanisms underlying vascular dysfunction...
January 31, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29440377/efficient-derivation-of-stable-primed-pluripotent-embryonic-stem-cells-from-bovine-blastocysts
#17
Yanina Soledad Bogliotti, Jun Wu, Marcela Vilarino, Daiji Okamura, Delia Alba Soto, Cuiqing Zhong, Masahiro Sakurai, Rafael Vilar Sampaio, Keiichiro Suzuki, Juan Carlos Izpisua Belmonte, Pablo Juan Ross
Embryonic stem cells (ESCs) are derived from the inner cell mass of preimplantation blastocysts. From agricultural and biomedical perspectives, the derivation of stable ESCs from domestic ungulates is important for genomic testing and selection, genome engineering, and modeling human diseases. Cattle are one of the most important domestic ungulates that are commonly used for food and bioreactors. To date, however, it remains a challenge to produce stable pluripotent bovine ESC lines. Employing a culture system containing fibroblast growth factor 2 and an inhibitor of the canonical Wnt-signaling pathway, we derived pluripotent bovine ESCs (bESCs) with stable morphology, transcriptome, karyotype, population-doubling time, pluripotency marker gene expression, and epigenetic features...
February 9, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29437173/human-brain-organoid-on-a-chip-to-model-prenatal-nicotine-exposure
#18
Yaqing Wang, Li Wang, Yujuan Zhu, Jianhua Qin
Nicotine has been recognized to trigger various neuronal disabilities in the fetal brain and long-lasting behavioral deficits in offspring. However, further understanding of fetal brain development under nicotine exposure is challenging due to the limitations of existing animal models. Here, we create a new brain organoid-on-a-chip system derived from human induced pluripotent stem cells (hiPSCs) that allows us to model neurodevelopmental disorders under prenatal nicotine exposure (PNE) at early stages. The brain organoid-on-a-chip system facilitates 3D culture, in situ neural differentiation, and self-organization of brain organoids under continuous perfused cultures in a controlled manner...
February 13, 2018: Lab on a Chip
https://www.readbyqxmd.com/read/29436856/chondroitinase-abc-for-neurological-recovery-after-acute-brain-injury-systematic-review-and-meta-analyses-of-preclinical-studies
#19
Chan Hee Koh, Savva Pronin, Mark Hughes
OBJECTIVES: Damage to critical brain regions causes deficits in important neurological functions. Chondroitinase ABC (ChABC) has been shown to promote neuroplasticity and may ameliorate neurological deficits caused by disease or trauma. This systematic review identifies and evaluates preclinical studies of ChABC as a treatment for acute brain injury. METHODS: Four databases were searched for studies relating to ChABC and brain or brain injuries. Controlled studies in mammals with acute brain injuries treated with ChABC were included in meta-analyses of neurobehavioural outcomes...
February 13, 2018: Brain Injury: [BI]
https://www.readbyqxmd.com/read/29436662/comparison-of-the-properties-of-neural-stem-cells-of-the-hippocampus-in-the-tree-shrew-and-rat-in%C3%A2-vitro
#20
Yuan-Dong Hu, Qiong Zhao, Xue-Rong Zhang, Liu-Lin Xiong, Zi-Bin Zhang, Piao Zhang, Rong-Ping Zhang, Ting-Hua Wang
Neural stem cells (NSCs) are characterized by the ability of self‑renewal and capacity to proliferate and produce new nervous tissue. NSCs are capable of differentiating to three lineages of neural cells, including neurons, oligodendrocytes and astrocytes. Furthermore, hippocampal NSCs transplantation can improve the neurological deficits associated with expression of cytokines. Therefore, to compare the properties of NSCs of tree shrews and rats in vitro, NSCs from tree shrews (tsNSCs) and rats f(rNSCs) were isolated...
February 12, 2018: Molecular Medicine Reports
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