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https://www.readbyqxmd.com/read/29334630/generation-of-an-integration-free-induced-pluripotent-stem-cell-line-csc-43j-from-a-patient-with-sporadic-parkinson-s-disease
#1
Ana Marote, Yuriy Pomeshchik, Stefano Goldwurm, Anna Collin, Nuno J Lamas, Luísa Pinto, António J Salgado, Laurent Roybon
An induced pluripotent stem cell (iPSC) line was generated from a 36-year-old patient with sporadic Parkinson's disease (PD). Skin fibroblasts were reprogrammed using the non-integrating Sendai virus technology to deliver OCT3/4, SOX2, c-MYC and KLF4 factors. The generated cell line (CSC-43) exhibits expression of common pluripotency markers, in vitro differentiation into three germ layers and normal karyotype. This iPSC line can be used to study the mechanisms underlying the development of PD.
January 4, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29334629/generation-of-a-human-induced-pluripotent-stem-cell-line-csc-42-from-a-patient-with-sporadic-form-of-parkinson-s-disease
#2
Ekaterina Savchenko, Ana Marote, Kaspar Russ, Anna Collin, Stefano Goldwurm, Laurent Roybon, Yuriy Pomeshchik
Skin fibroblasts were collected from a 44-year-old patient with sporadic case of Parkinson's disease (PD). The non-integrating Sendai virus vector encoding OCT3/4, SOX2, c-MYC and KLF4 was used to reprogram fibroblasts into induced pluripotent stem cells (iPSCs). Generated iPSCs had normal karyotypes, expressed common stem cell markers, and were capable of differentiating into all three germ layers. Generated line could be used for PD modeling to understand the mechanisms that influence the disorder.
January 4, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29331938/generation-of-a-human-induced-pluripotent-stem-cell-line-csc-40-from-a-parkinson-s-disease-patient-with-a-pink1-p-q456x-mutation
#3
Kaspar Russ, Ana Marote, Ekaterina Savchenko, Anna Collin, Stefano Goldwurm, Yuriy Pomeshchik, Laurent Roybon
Parkinson's disease (PD) is a neurodegenerative disease with unknown etiology. Here we show the generation of an induced pluripotent stem cell (iPSC) line, named CSC-40, from dermal fibroblasts obtained from a 59-year-old male patient with a homozygous p.Q456X mutation in the PTEN-induced putative kinase 1 (PINK/PARK6) gene and a confirmed diagnosis of PD, which could be used to model familial PD. A non-integrating Sendai virus-based delivery of the reprogramming factors OCT3/4, SOX2, c-MYC and KLF4 was employed...
January 4, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29321315/paramyxovirus-v-proteins-interact-with-the-rig-i-trim25-regulatory-complex-and-inhibit-rig-i-signaling
#4
Maria T Sanchez-Aparicio, Leighland J Feinman, Adolfo García-Sastre, Megan L Shaw
Paramyxovirus V proteins are known antagonists of the RIG-I-like receptor (RLR)-mediated interferon induction pathway, interacting with and inhibiting the RLR MDA5. We report interactions between the Nipah virus V protein and both the RIG-I regulatory protein, TRIM25, and RIG-I. We also observed interactions between these host proteins and the V proteins of measles virus, Sendai virus and parainfluenza virus. These interactions are mediated by the conserved C-terminal domain of the V protein, which binds to the tandem CARDs of RIG-I (the region of TRIM25-ubiquitination) and to the SPRY domain of TRIM25, which mediates TRIM25 interaction with the RIG-I CARDs...
January 10, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29304399/establishment-of-induced-pluripotent-stem-cell-line-zzui009-a-from-an-alzheimer-s-disease-patient-carrying-a-psen1-gene-mutation
#5
Yanlin Wang, Na Jing, Linlin Su, Changhe Shi, Pei Zhang, Zhilei Wang, Huifang Sun, Jing Yang, Yutao Liu, Xuejun Wen, Jin Zhang, Shoutao Zhang, Yuming Xu
Skin fibroblasts were obtained from a 42-year-old Alzheimer's disease (AD) patient carrying mutations in the PSEN1 gene. An iPSC line was successfully established using the Sendai-virus (SeV) delivery system. The patient-specific iPSCs were free of genomically integrated reprogramming genes, had the specific mutation, expressed the expected pluripotency markers, and had the potential to differentiate into cells of all three germ layers. Our model might offer a robust platform for further study of the pathomechanism of this disease as well as drug testing and gene therapy studies...
December 12, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29301940/the-nlr-family-pyrin-domain-containing-11-protein-contributes-to-the-regulation-of-inflammatory-signalling
#6
Kornelia Ellwanger, Emily Becker, Ioannis Kienes, Anna Sowa, Yvonne Postma, Yamel Cardona Gloria, Alexander N R Weber, Thomas A Kufer
Mammalian NLR proteins contribute to the regulation and induction of innate and adaptive immunity in mammals although the function of about half of the currently identified NLR proteins remains poorly characterized. Here we analysed the function of the primate-specific NLRP11 gene product. We show that NLRP11 is highly expressed in immune cells, including myeloid cells, B cells and some B cell lymphoma lines. Overexpression of NLRP11 in human cells did not trigger key innate immune signalling pathways including NF-κB and type I interferon responses...
January 4, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29276141/direct-in%C3%A2-vivo-reprogramming-with-sendai-virus-vectors-improves-cardiac-function-after-myocardial-infarction
#7
Kazutaka Miyamoto, Mizuha Akiyama, Fumiya Tamura, Mari Isomi, Hiroyuki Yamakawa, Taketaro Sadahiro, Naoto Muraoka, Hidenori Kojima, Sho Haginiwa, Shota Kurotsu, Hidenori Tani, Li Wang, Li Qian, Makoto Inoue, Yoshinori Ide, Junko Kurokawa, Tsunehisa Yamamoto, Tomohisa Seki, Ryo Aeba, Hiroyuki Yamagishi, Keiichi Fukuda, Masaki Ieda
Direct cardiac reprogramming holds great promise for regenerative medicine. We previously generated directly reprogrammed induced cardiomyocyte-like cells (iCMs) by overexpression of Gata4, Mef2c, and Tbx5 (GMT) using retrovirus vectors. However, integrating vectors pose risks associated with insertional mutagenesis and disruption of gene expression and are inefficient. Here, we show that Sendai virus (SeV) vectors expressing cardiac reprogramming factors efficiently and rapidly reprogram both mouse and human fibroblasts into integration-free iCMs via robust transgene expression...
January 4, 2018: Cell Stem Cell
https://www.readbyqxmd.com/read/29274549/lymphoblastoids-cell-lines-derived-ipsc-line-from-a-26-year-old-myotonic-dystrophy-type-1-patient-carrying-ctg-200-expansion-in-the-dmpk-gene-chuqi001-a
#8
Laurie Martineau, Véronique Racine, Siham Ait Benichou, Jack Puymirat
Human immortalized Epstein-Barr virus (EBV) lymphoblastoids cells line (LCLs) from a 26-year- old male affected by an adult form of myotonic dystrophy type 1 (DM1) disease and carrying 200 CTG repeats mutation in the blood was used to generate induced pluripotent stem cells (iPSCs) using the Sendai virus expressing KLF4, OCT4, SOX2 and C-MYC. The resulting iPSCs were EBV free, expressed the pluripotency markers, could be differentiated into the three germ layers in vitro, had a normal karyotype, and retained the genetic DM1 mutation...
December 16, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29244204/respiratory-viral-infections-and-atopic-development-from-possible-mechanisms-to-advances-in-treatment
#9
REVIEW
Lisa M Martorano, Mitchell H Grayson
Atopic sensitization and allergic diseases are increasing in modernized countries. These diseases affect millions of individuals, but the mechanisms behind their development are not fully understood. One hypothesis relates to early life respiratory viral infections driving the development of atopic disease including asthma. This review presents the current state of the field, focusing on epidemiologic data supporting a role for early life respiratory viruses in the development of specific IgE, both against aeroallergens and the respiratory virus...
December 15, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29237838/a-single-amino-acid-substitution-within-the-paramyxovirus-sendai-virus-nucleoprotein-is-a-critical-determinant-for-production-of-ifn-beta-inducing-copyback-type-defective-interfering-genomes
#10
Asuka Yoshida, Ryoko Kawabata, Tomoyuki Honda, Kouji Sakai, Yasushi Ami, Takemasa Sakaguchi, Takashi Irie
One of the first defenses against infecting pathogens is the innate immune system activated by cellular recognition of pathogen-associated molecular patterns (PAMPs). Although virus-derived RNA species, especially copyback (cb)-type defective-interfering (DI) genomes, have been shown to serve as real PAMPs, which strongly induce interferon (IFN)-beta during mononegavirus infection, the mechanisms underlying DI generation remain unclear. Here, for the first time, we identified a single amino acid substitution causing production of cbDI genomes by successful isolation of two distinct types of viral clones with cbDI-producing and cbDI-non-producing phenotypes, from the stock Sendai virus (SeV) strain Cantell, which is widely used in a number of studies on anti-viral innate immunity as a representative IFN-beta-inducing virus...
December 13, 2017: Journal of Virology
https://www.readbyqxmd.com/read/29234123/dusp1-regulates-apoptosis-and-cell-migration-but-not-the-jip1-protected-cytokine-response-during-respiratory-syncytial-virus-and-sendai-virus-infection
#11
Alexa C Robitaille, Elise Caron, Nicolas Zucchini, Espérance Mukawera, Damien Adam, Mélissa K Mariani, Anaïs Gélinas, Audray Fortin, Emmanuelle Brochiero, Nathalie Grandvaux
The host antiviral response involves the induction of interferons and proinflammatory cytokines, but also the activation of cell death pathways, including apoptosis, to limit viral replication and spreading. This host defense is strictly regulated to eliminate the infection while limiting tissue damage that is associated with virus pathogenesis. Post-translational modifications, most notably phosphorylation, are key regulators of the antiviral defense implying an important role of protein phosphatases. Here, we investigated the role of the dual-specificity phosphatase 1 (DUSP1) in the host defense against human respiratory syncytial virus (RSV), a pathogenic virus of the Pneumoviridae family, and Sendai virus (SeV), a model virus being developed as a vector for anti-RSV vaccine...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29201911/transmissible-gastroenteritis-virus-papain-like-protease-1-antagonizes-production-of-interferon-%C3%AE-through-its-deubiquitinase-activity
#12
Xiaoliang Hu, Jin Tian, Hongtao Kang, Dongchun Guo, Jiasen Liu, Dafei Liu, Qian Jiang, Zhijie Li, Juanjuan Qu, Liandong Qu
Coronaviruses (CoVs), such as human coronavirus NL63 (HCoV-NL63), severe acute respiratory syndrome CoV (SARS-CoV), murine hepatitis virus (MHV), porcine epidemic diarrhea virus (PEDV), and Middle East Respiratory Syndrome Coronavirus (MERS-CoV), encode papain-like (PL) proteases that inhibit Sendai virus- (SeV-) induced interferon (IFN-β) production. Recently, the crystal structure of transmissible gastroenteritis virus (TGEV) PL1 has been solved, which was similar to that of SARS-CoV PL2pro, which may antagonize host innate immunity...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29189674/concurrent-community-transmission-of-enterovirus-d68-with-human-rhinoviruses-and-respiratory-syncytial-virus-among-children-in-sendai-japan
#13
Takaya Metoki, Michiko Okamoto, Akira Suzuki, Setsuko Kitaoka, Hiroki Miyabayashi, Yuka Rokugo, Ryoichi Onuma, Suguru Ohmiya, Ko Sato, Hidekazu Nishimura, Hitoshi Oshitani, Satoru Kumaki, Rie Noguchi, Taiki Sato, Yohei Watanabe
BACKGROUND: In the autumn of 2015, we experienced a surge in the number of pediatric cases of wheeze in our hospital, which was suspected to be caused by enterovirus (EV)-D68 transmission in the community. Thus, we implemented an ad hoc retrospective surveillance for EV-D68. METHODS: Patients aged < 15 years old with acute respiratory infection were eligible for inclusion in this study. All enrolled patients underwent virus detection test. Additionally, neutralization tests (NTs) were performed using the stored serum samples of the enrolled patients to compare the antigenicity of the virus isolated in this study with that isolated in 2010 and evaluate the anti-EV-D68 antibody prevalence...
November 17, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29174977/generation-of-an-induced-pluripotent-stem-cell-line-from-a-39-year-old-female-patient-with-severe-to-profound-non-syndromic-sensorineural-hearing-loss-and-a-a1555g-mutation-in-the-mitochondrial-mtrnr1-gene
#14
Yu-Hung Hsu, Yu-Ting Wu, Ching-Ying Huang, Ming-Ching Ho, Yu-Che Cheng, Shih-Han Syu, Huai-En Lu, Yen-Chun Chen, Chia-Ling Tsai, Hung-Ching Lin, Yau-Huei Wei, Yi-Chao Hsu, Patrick C H Hsieh
Sensorineural hearing loss (SNHL) is a prevalent form of deafness commonly arising from damage to the cochlear sensory hair cells and degeneration of the spiral ganglion neurons. In this study, Sendai virus was used to generate an induced pluripotent stem cell (iPSC) line from a 39-year-old female patient diagnosed with severe-to-profound, non-syndromic SNHL. The patient also carries a A1555G mutation in the mitochondrial 12S ribosome RNA gene (MTRNR1). This iPSC line was verified to express pluripotent markers, possess normal karyotype, harbor the specific mutation and demonstrated the capacity to differentiate into three germ layers...
November 8, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29168031/neural-cells-generated-from-human-induced-pluripotent-stem-cells-as-a-model-of-cns-involvement-in-mucopolysaccharidosis-type-ii
#15
Jitka Rybová, Jana Ledvinová, Jakub Sikora, Ladislav Kuchař, Robert Dobrovolný
Mucopolysaccharidosis type II (MPSII) is a rare X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene (IDS, Xq28). MPSII is characterized by skeletal deformities, hearing loss, airway obstruction, hepatosplenomegaly, cardiac valvular disease, and progressive neurological impairment. At the cellular level, IDS deficiency leads to lysosomal storage of glycosaminoglycans (GAGs), dominated by accumulation of dermatan and heparan sulfates. Human induced pluripotent stem cells (iPSC) represent an alternative system that complements the available MPSII murine model...
November 22, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29164800/the-generation-of-human-%C3%AE-%C3%AE-t-cell-derived-induced-pluripotent-stem-cells-from-whole-peripheral-blood-mononuclear-cell-culture
#16
Daisuke Watanabe, Michiyo Koyanagi-Aoi, Mariko Taniguchi-Ikeda, Yukiko Yoshida, Takeshi Azuma, Takashi Aoi
γδT cells constitute a small proportion of lymphocytes in peripheral blood. Unlike αβT cells, the anti-tumor activities are exerted through several different pathways in a MHC-unrestricted manner. Thus, immunotherapy using γδT cells is considered to be effective for various types of cancer. Occasionally, however, ex vivo expanded cells are not as effective as expected due to cell exhaustion. To overcome the issue of T-cell exhaustion, researchers have generated induced pluripotent stem cells (iPSCs) that harbor the same T-cell receptor (TCR) genes as their original T-cells, which provide nearly limitless sources for antigen-specific cytotoxic T lymphocytes (CTLs)...
January 2018: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29162867/broad-spectrum-antiviral-agents-secreted-phospholipase-a2-targets-viral-envelope-lipid-bilayers-derived-from-the-endoplasmic-reticulum-membrane
#17
Ming Chen, Chie Aoki-Utsubo, Masanori Kameoka, Lin Deng, Yutaka Terada, Wataru Kamitani, Kei Sato, Yoshio Koyanagi, Makoto Hijikata, Keiko Shindo, Takeshi Noda, Michinori Kohara, Hak Hotta
Hepatitis C virus (HCV), dengue virus (DENV) and Japanese encephalitis virus (JEV) belong to the family Flaviviridae. Their viral particles have the envelope composed of viral proteins and a lipid bilayer acquired from budding through the endoplasmic reticulum (ER). The phospholipid content of the ER membrane differs from that of the plasma membrane (PM). The phospholipase A2 (PLA2) superfamily consists of a large number of members that specifically catalyse the hydrolysis of phospholipids at a particular position...
November 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29156377/establishment-of-induced-pluripotent-stem-cell-line-zzui010-a-from-an-alzheimer-s-disease-patient-carrying-an-app-gene-mutation
#18
Zhilei Wang, Pei Zhang, Yanlin Wang, Changhe Shi, Na Jing, Huifang Sun, Jing Yang, Yutao Liu, Xuejun Wen, Jin Zhang, Shoutao Zhang, Yuming Xu
Alzheimer's disease (AD) is one of the most common neurodegenerative disorders. Previous studies have identified mutations in several genes, such as amyloid precursor protein (APP), presenilin-1 (PSEN1), and presenilin-2 (PSEN2), in patients with early-onset (<65years) familial AD. Recently, a patient with an APP gene mutation was identified; the dermal fibroblasts of the patient were obtained and a line of induced pluripotent stem cells (iPSCs) was successfully generated using the Sendai-virus (SeV) delivery system...
November 3, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29154227/generation-of-integration-free-induced-pluripotent-stem-cell-line-njmui001-a-from-a-phenylketonuria-patient
#19
Tianhui Xu, Dong Liang, Jingjing Zhang, Xiuqing Ji, Huanran Hu, Yun Sun, Tao Jiang, Xia Wang, Ping Hu, Zhengfeng Xu
PKU is a prevalent type of inherited metabolic disease, caused by the defective phenylalanine metabolism. In most PKU cases, mutations in the PAH gene could be found. Dysfunction of this hepatic enzyme will lead to diverse clinical symptoms due to a failure in converting phenylalanine into tyrosine. Here, we report an integration-free human induced pluripotent stem cell line (NJMUi001-A) generated from peripheral blood mononuclear cells of a PKU patient by using Sendai virus. This iPS cell line has characteristics of pluripotent stem cells and can be used as a useful tool for the investigation of this inherited metabolic disease...
November 10, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29136563/generation-of-human-ipsc-line-from-a-patient-with-laterality-defects-and-associated-congenital-heart-anomalies-carrying-a-dand5-missense-alteration
#20
Fernando Cristo, José M Inácio, Graça Rosas, Isabel Marques Carreira, Joana Barbosa Melo, Luís Pereira de Almeida, Patrícia Mendes, Duarte Saraiva Martins, José Maio, Rui Anjos, José A Belo
A human iPSC line was generated from exfoliated renal epithelial (ERE) cells of a patient affected with Congenital Heart Disease (CHD) and Laterality Defects carrying tshe variant p.R152H in the DAND5 gene. The transgene-free iPSCs were generated with the human OSKM transcription factor using the Sendai-virus reprogramming system. The established iPSC line had the specific heterozygous alteration, a stable karyotype, expressed pluripotency markers and generated embryoid bodies that can differentiate towards the three germ layers in vitro...
October 31, 2017: Stem Cell Research
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