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https://www.readbyqxmd.com/read/28717411/skeletal-muscle-generated-from-induced-pluripotent-stem-cells-induction-and-application
#1
REVIEW
Yuko Miyagoe-Suzuki, Shin'ichi Takeda
Human induced pluripotent stem cells (hiPS cells or hiPSCs) can be derived from cells of patients with severe muscle disease. If skeletal muscle induced from patient-iPSCs shows disease-specific phenotypes, it can be useful for studying the disease pathogenesis and for drug development. On the other hand, human iPSCs from healthy donors or hereditary muscle disease-iPSCs whose genomes are edited to express normal protein are expected to be a cell source for cell therapy. Several protocols for the derivation of skeletal muscle from human iPSCs have been reported to allow the development of efficient treatments for devastating muscle diseases...
June 26, 2017: World Journal of Stem Cells
https://www.readbyqxmd.com/read/28717316/induced-pluripotent-stem-cell-a-headway-in-reprogramming-with-promising-approach-in-regenerative-biology
#2
REVIEW
N Rawat, M K Singh
Since the embryonic stem cells have knocked the doorsteps, they have proved themselves in the field of science, research, and medicines, but the hovered restrictions confine their application in human welfare. Alternate approaches used to reprogram the cells to the pluripotent state were not up to par, but the innovation of induced pluripotent stem cells (iPSCs) paved a new hope for the researchers. Soon after the discovery, iPSCs technology is undergoing renaissance day by day, i.e., from the use of genetic material to recombinant proteins and now only chemicals are employed to convert somatic cells to iPSCs...
June 2017: Veterinary World
https://www.readbyqxmd.com/read/28716732/lysophosphatidic-acid-signaling-regulates-the-klf9-ppar%C3%AE-axis-in-human-induced-pluripotent-stem-cell-derived-neurons
#3
Tamotsu Tsukahara, Shuhei Yamagishi, Yoshikazu Matsuda, Hisao Haniu
Lysophosphatidic acid (LPA) is a lipid signaling molecule that plays several significant roles in the nervous system during development and injury. In this study, we differentiated human induced pluripotent stem cells (iPSCs) into neurons as an in vitro model to examine the specific effects of LPA. We demonstrated that LPA activates peroxisome proliferator-activated receptor gamma (PPARγ), a ligand-activated nuclear receptor, as well as its cognate receptor LPA1 on human iPSC-derived neurons to enhance proliferation and neurite outgrowth...
July 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28716551/application-of-human-induced-pluripotent-stem-cells-to-model-fibrodysplasia-ossificans-progressiva
#4
Emilie Barruet, Edward C Hsiao
Fibrodysplasia ossificans progressiva (FOP) is a genetic condition characterized by massive heterotopic ossification. FOP patients with mutations in the Activin A type I receptor (ACVR1), a bone morphogenetic protein (BMP) receptor. FOP is a progressive and debilitating disease characterized by bone formation flares that often occur after trauma. Since it is often difficult or impossible to obtain large amounts of tissue from human donors due to the risks of inciting more heterotopic bone formation, human induced pluripotent stem cells (hiPSCs) provide an attractive source for establishing in vitro disease models and for applications in drug screening...
July 14, 2017: Bone
https://www.readbyqxmd.com/read/28713933/mir%C3%A2-146b%C3%A2-5p-promotes-the-neural-conversion-of-pluripotent-stem-cells-by-targeting-smad4
#5
Nianping Zhang, Ying Lyu, Xuebing Pan, Liping Xu, Aiguo Xuan, Xiaosong He, Wandan Huang, Dahong Long
Pluripotent stem cells (PSCs) are regarded as potential sources that provide specific neural cells for cell therapy in some nervous system diseases. However, the mechanisms underlying the neural differentiation of PSCs remain largely unknown. MicroRNAs (miRNAs or miRs) are a class of small non‑pro-tein-coding RNAs that act as critical regulatory molecules in many cellular processes. In this study, we found that miR‑146b‑5p expression was markedly increased following the neural induction of mouse embryonic stem cells (ESCs) or induced PSCs (iPSCs)...
July 11, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28710827/comparison-of-non-coding-rnas-in-exosomes-and-functional-efficacy-of-human-embryonic-stem-cell-versus-induced-pluripotent-stem-cell-derived-cardiomyocytes
#6
Won Hee Lee, Wenyi Chen, Ning-Yi Shao, Dan Xiao, Xulei Qin, Natalie Baker, Hye Ryeong Michelle Bae, Praveen Shukla, Haodi Wu, Kazuki Kodo, Sang-Ging Ong, Joseph C Wu
BACKGROUND: Both human embryonic stem cell-derived cardiomyocytes (ESC-CMs) and human induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) can serve as unlimited cell sources for cardiac regenerative therapy. However, the functional equivalency between human ESC-CMs and iPSC-CMs for cardiac regenerative therapy has not been demonstrated. Here we performed a head-to-head comparison of ESC-CMs and iPSC-CMs in their ability to restore cardiac function in a rat myocardial infarction (MI) model as well as their exosomal secretome...
July 14, 2017: Stem Cells
https://www.readbyqxmd.com/read/28710685/pericytes-extend-survival-of-als-sod1-mice-and-induce-the-expression-of-antioxidant-enzymes-in-the-murine-model-and-in-ipscs-derived-neuronal-cells-from-an-als-patient
#7
Giuliana Castello Coatti, Miriam Frangini, Marcos C Valadares, Juliana Plat Gomes, Natalia O Lima, Natale Cavaçana, Amanda F Assoni, Mayra V Pelatti, Alexander Birbrair, Antonio Carlos Pedroso de Lima, Julio M Singer, Francisco Marcelo M Rocha, Giovani Loiola Da Silva, Mario Sergio Mantovani, Lucia Inês Macedo-Souza, Merari F R Ferrari, Mayana Zatz
Amyotrophic Lateral Sclerosis (ALS) is one of the most common adult-onset motor neuron disease causing a progressive, rapid and irreversible degeneration of motor neurons in the cortex, brain stem and spinal cord. No effective treatment is available and cell therapy clinical trials are currently being tested in ALS affected patients. It is well known that in ALS patients, approximately 50% of pericytes from the spinal cord barrier are lost. In the central nervous system, pericytes act in the formation and maintenance of the blood-brain barrier, a natural defense that slows the progression of symptoms in neurodegenerative diseases...
July 14, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28710467/human-ipsc-derived-cardiomyocytes-cultured-in-3d-engineered-heart-tissue-show-physiological-upstroke-velocity-and-sodium-current-density
#8
Marc D Lemoine, Ingra Mannhardt, Kaja Breckwoldt, Maksymilian Prondzynski, Frederik Flenner, Bärbel Ulmer, Marc N Hirt, Christiane Neuber, András Horváth, Benjamin Kloth, Hermann Reichenspurner, Stephan Willems, Arne Hansen, Thomas Eschenhagen, Torsten Christ
Human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CM) are a promising tool for drug testing and modelling genetic disorders. Abnormally low upstroke velocity is a current limitation. Here we investigated the use of 3D engineered heart tissue (EHT) as a culture method with greater resemblance to human heart tissue in comparison to standard technique of 2D monolayer (ML) format. INa was measured in ML or EHT using the standard patch-clamp technique. INa density was ~1.8 fold larger in EHT (-18...
July 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28704293/analysis-of-neural-crest-cells-from-charcot-marie-tooth-disease-patients-demonstrates-disease-relevant-molecular-signature
#9
Fukiko Kitani-Morii, Keiko Imamura, Takayuki Kondo, Ryo Ohara, Takako Enami, Ran Shibukawa, Takuya Yamamoto, Kazuya Sekiguchi, Junya Toguchida, Toshiki Mizuno, Masanori Nakagawa, Haruhisa Inoue
Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy. The majority of CMT is demyelinating type (demyelinating CMT) caused by Schwann cell involvement. Although a large number of genes responsible for demyelinating CMT have been found, the common molecular target of the pathophysiology caused by these different genes in demyelinating CMT is still unknown. We generated induced pluripotent stem cells (iPSCs) from healthy controls and patients with demyelinating CMT caused by duplication in peripheral myelin protein 22 kDa (PMP22) or point mutations in myelin protein zero (MPZ) or early growth response 2 (EGR2)...
July 12, 2017: Neuroreport
https://www.readbyqxmd.com/read/28702161/bio-collections-in-autism-research
#10
REVIEW
Jamie Reilly, Louise Gallagher, June L Chen, Geraldine Leader, Sanbing Shen
Autism spectrum disorder (ASD) is a group of complex neurodevelopmental disorders with diverse clinical manifestations and symptoms. In the last 10 years, there have been significant advances in understanding the genetic basis for ASD, critically supported through the establishment of ASD bio-collections and application in research. Here, we summarise a selection of major ASD bio-collections and their associated findings. Collectively, these include mapping ASD candidate genes, assessing the nature and frequency of gene mutations and their association with ASD clinical subgroups, insights into related molecular pathways such as the synapses, chromatin remodelling, transcription and ASD-related brain regions...
2017: Molecular Autism
https://www.readbyqxmd.com/read/28701538/differential-expression-of-long-term-potentiation-among-identified-inhibitory-inputs-to-dopamine-neurons
#11
DeNard V Simmons, Alyssa K Petko, Carlos Antonio Paladini
The in vivo firing pattern of ventral tegmental area (VTA) dopamine neurons is controlled by GABA afferents originating primarily from the nucleus accumbens (NAc), rostromedial tegmental nucleus (RMTg), and from local GABA neurons within the VTA. Although different forms of plasticity have been observed from GABA inputs to VTA dopamine neurons - one dependent on cyclic GMP synthesis, the other dependent on adenylyl cyclase activation - it is unknown whether plasticity is differentially expressed in each. Using an optogenetic strategy, we show that identified inhibitory postsynaptic currents (IPSCs) from local VTA GABA neurons and NAc afferents exhibit a cyclic GMP-dependent long-term potentiation (LTP) that is capable of inhibiting the firing activity of dopamine neurons...
July 12, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28700940/in%C3%A2-vitro-modeling-using-ciliopathy-patient-derived-cells-reveals-distinct-cilia-dysfunctions-caused-by-cep290-mutations
#12
Hiroko Shimada, Quanlong Lu, Christine Insinna-Kettenhofen, Kunio Nagashima, Milton A English, Elizabeth M Semler, Jacklyn Mahgerefteh, Artur V Cideciyan, Tiansen Li, Brian P Brooks, Meral Gunay-Aygun, Samuel G Jacobson, Tiziana Cogliati, Christopher J Westlake, Anand Swaroop
Mutations in CEP290, a transition zone protein in primary cilia, cause diverse ciliopathies, including Leber congenital amaurosis (LCA) and Joubert-syndrome and related disorders (JSRD). We examined cilia biogenesis and function in cells derived from CEP290-LCA and CEP290-JSRD patients. CEP290 protein was reduced in LCA fibroblasts with no detectable impact on cilia; however, optic cups derived from induced pluripotent stem cells (iPSCs) of CEP290-LCA patients displayed less developed photoreceptor cilia. Lack of CEP290 in JSRD fibroblasts resulted in abnormal cilia and decreased ciliogenesis...
July 11, 2017: Cell Reports
https://www.readbyqxmd.com/read/28699384/preclinical-safety-evaluation-of-allogeneic-ips-cell-based-therapy-in-a-swine-model-of-myocardial-infarction
#13
Carolina Gálvez-Montón, Carolina Soler-Botija, Oriol Iborra-Egea, Idoia Diaz-Guemes, Mercè Martí, Olalla Iglesias-Garcia, Cristina Prat-Vidal, Verónica Crisóstomo, Aida Llucià-Valldeperas, Isaac Perea-Gil, Santiago Roura, Francisco Miguel Sanchez Margallo, Ángel Raya, Antoni Bayés-Genís
The combination of biomatrices and induced pluripotent stem cell (iPSC) derivatives to aid repair and myocardial scar formation may soon become a reality for cardiac regenerative medicine. However, the tumor risk associated to residual undifferentiated cells remains an important safety concern of iPSC-based therapies. This concern is not satisfactorily addressed in xenotransplantation, which require immune suppression of the transplanted animal. Here, we assessed the safety of transplanting undifferentiated iPSC in an allogeneic setting...
July 12, 2017: Tissue Engineering. Part C, Methods
https://www.readbyqxmd.com/read/28695746/improvement-of-hepatogenic-differentiation-of-ips-cells-on-an-aligned-polyethersulfone-compared-to-random-nanofibers
#14
Maryam Mahmoodinia Maymand, Hamid Reza Soleimanpour-Lichaei, Abdolreza Ardeshirylajimi, Masoud Soleimani, Seyed Ehsan Enderami, Shahrzad Nojehdehi, Farkhondeh Behjati, Maryam Kabir Salmani
The application of stem cells holds great promises in cell and tissue transplants. This study was designed to compare the hepatogenic differentiation of iPSCs on aligned PES/COL versus random. Aligned and random PES/COL nanofibrus scaffolds were fabricated by electrospining and their surface modified through plasma treatment and collagen coating. The scaffolds were characterized using scanning electron microscopy (SEM) and ATR-FTIR. Morphology and biochemical activities of the differentiated hepatocyte-like cells (HLCs) were examined after 5 and 20 days of differentiation...
July 11, 2017: Artificial Cells, Nanomedicine, and Biotechnology
https://www.readbyqxmd.com/read/28695744/genome-editing-the-breakthrough-technology-for-inherited-retinal-disease
#15
Andrew J Smith, Stephen P Carter, Breandán N Kennedy
Genetic alterations resulting in a dysfunctional retinal pigment epithelium and/or degenerating photoreceptors cause impaired vision. These juxtaposed cells in the retina of the posterior eye are crucial for the visual cycle or phototransduction. Deficits in these biochemical processes perturb neural processing of images capturing the external environment. Notably, there is a distinct lack of clinically approved pharmacological, cell- or gene-based therapies for inherited retinal disease. Gene editing technologies are rapidly advancing as a realistic therapeutic option...
July 11, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28689993/mapt-genetic-variation-and-neuronal-maturity-alter-isoform-expression-affecting-axonal-transport-in-ipsc-derived-dopamine-neurons
#16
Joel E Beevers, Mang Ching Lai, Emma Collins, Heather D E Booth, Federico Zambon, Laura Parkkinen, Jane Vowles, Sally A Cowley, Richard Wade-Martins, Tara M Caffrey
The H1 haplotype of the microtubule-associated protein tau (MAPT) locus is genetically associated with neurodegenerative diseases, including Parkinson's disease (PD), and affects gene expression and splicing. However, the functional impact on neurons of such expression differences has yet to be fully elucidated. Here, we employ extended maturation phases during differentiation of induced pluripotent stem cells (iPSCs) into mature dopaminergic neuronal cultures to obtain cultures expressing all six adult tau protein isoforms...
July 3, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28688751/electro-mechanical-conditioning-of-human-ipsc-derived-cardiomyocytes-for-translational-research
#17
REVIEW
Katharina Kroll, Mamta Chabria, Ken Wang, Fabian Häusermann, Franz Schuler, Liudmila Polonchuk
RATIONALE: Impaired maturation of human iPSC-derived cardiomyocytes (hiPSC-CMs) currently limits their use in experimental research and further optimization is required to unlock their full potential. OBJECTIVE: To push hiPSC-CMs towards maturation, we recapitulated the intrinsic cardiac properties by electro-mechanical stimulation and explored how these mimetic biophysical cues interplay and influence the cell behaviour. METHODS AND RESULTS: We introduced a novel device capable of applying synchronized electrical and mechanical stimuli to hiPSC-CM monolayers cultured on a PDMS membrane and evaluated effects of conditioning on cardiomyocyte structure and function...
July 5, 2017: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/28687556/spatiotemporal-proteomic-profiling-of-human-cerebral-development
#18
Ugljesa Djuric, Deivid C Rodrigues, Ihor Batruch, James Ellis, Patrick Shannon, Phedias Diamandis
Mass spectrometry (MS) analysis of human post-mortem central nervous system (CNS) tissue and induced pluripotent stem cell (iPSC)-based directed differentiations offer complementary avenues to define protein signatures of neurodevelopment. Methodological improvements of formalin-fixed, paraffin-embedded (FFPE) protein isolation now enable widespread proteomic analysis of well-annotated archival tissue samples in the context of development and disease. Here, we utilize a shotgun label-free quantification (LFQ) MS method to profile magnetically enriched human cortical neurons and neural progenitor cells (NPCs) derived from iPSCs...
July 7, 2017: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/28686863/the-evolving-roles-of-human-ipsc-derived-cardiomyocytes-in-drug-safety-and-discovery
#19
Gary Gintant, Bernard Fermini, Norman Stockbridge, David Strauss
Nonclinical studies of drug effects with human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) provide new possibilities for evaluating drug safety and efficacy. The Comprehensive In Vitro Proarrhythmia Assay (CiPA) paradigm provides lessons from the cardiac field that also apply to drug studies with other stem cell-based assays.
July 6, 2017: Cell Stem Cell
https://www.readbyqxmd.com/read/28686012/efficient-encapsulation-and-sustained-release-of-basic-fibroblast-growth-factor-in-nanofilm-extension-of-the-feeding-cycle-of-human-induced-pluripotent-stem-cell-culture
#20
Uiyoung Han, Hee Ho Park, Yu Jin Kim, Tai Hyun Park, Ju Hyun Park, Jinkee Hong
Basic fibroblast growth factor (bFGF) has an established pivotal function in biomedical engineering, especially for the human pluripotent stem cells (hPSCs). However, the limitation of bFGF is the ease of denaturation under normal physiological conditions, inducing loss of its activity. In this study, we designed multi-trilayered nanofilm composed of a repeating polycation/polyanion/bFGF structure, which has high loading efficiency and short build-up time. We also investigated that the loading and release of bFGF from the nanofilm with two parameters (counter-polyanion and film architectures)...
July 7, 2017: ACS Applied Materials & Interfaces
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