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GSE111106 Rostrocaudal Areal Patterning of Human PSC-Derived Cortical Neurons by FGF8 Signaling

In this study, we recapitulated cortical R-C patterning in human pluripotent stem cell (PSC) cultures. Modulation of FGF8 signaling appropriately regulated the R-C markers, and the patterns of global gene expression resembled those of the corresponding areas of in vivo human fetal brains. Furthermore, we demonstrated the utility of this culture system in modeling the area-specific forebrain phenotypes (presumptive upper motor neuron (UMN) phenotypes) of amyotrophic lateral sclerosis (ALS). We anticipate that our culture system will contribute to studies of human neurodevelopment and neurological disease modeling.
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Homo sapiens Source Type: research

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AbstractMutations in fused in sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS). FUS is a multifunctional protein involved in the biogenesis and activity of several types of RNAs, and its role in the pathogenesis of ALS may involve both direct effects of disease-associated mutations through gain- and loss-of-function mechanisms and indirect effects due to the cross talk between different classes of FUS-dependent RNAs. To explore how FUS mutations impinge on motor neuron-specific RNA-based circuitries, we performed transcriptome profiling of small and long RNAs of motor neurons (MNs) derived from mouse embryonic stem ...
Source: Molecular Neurobiology - Category: Neurology Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is incurable and devastating. A dearth of therapies has galvanized experimental focus onto the cellular and molecular mechanisms that both initiate and subsequently drive motor neuron degeneration. A traditional view of ALS pathogenesis posits that disease‐specific injury to a subtype of neurons is mechanistically cell‐autonomous. This “neuron‐centric” view has biased past research efforts. However, a wealth of accumulating evidence now strongly implicates non‐neuronal cells as being major determinants of ALS. Although animal models have proven invaluable in ...
Source: Stem Cells - Category: Stem Cells Authors: Tags: Embryonic Stem Cells/Induced Pluripotent Stem Cells Source Type: research
This article is protected by copyright. All rights reserved. “Intricate and self‐perpetuating cellular interplay underlies amyotrophic lateral sclerosis (ALS). Disease stage is a key determinant of cell type‐specific molecular mechanisms”.
Source: Stem Cells - Category: Stem Cells Authors: Tags: Embryonic Stem Cells/Induced Pluripotent Stem Cells Source Type: research
Publication date: December 2017 Source:Stem Cell Research, Volume 25 Author(s): Francesca Sironi, Antonio Vallarola, Martina Bruna Violatto, Laura Talamini, Mattia Freschi, Roberta De Gioia, Chiara Capelli, Azzurra Agostini, Davide Moscatelli, Massimo Tortarolo, Paolo Bigini, Martino Introna, Caterina Bendotti Stem cell therapy is considered a promising approach in the treatment of amyotrophic lateral sclerosis (ALS) and mesenchymal stem cells (MSCs) seem to be the most effective in ALS animal models. The umbilical cord (UC) is a source of highly proliferating fetal MSCs, more easily collectable than other MSCs. Recently ...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
This article is protected by copyright. All rights reserved
Source: Journal of Cellular Physiology - Category: Cytology Authors: Tags: ORIGINAL RESEARCH ARTICLE Source Type: research
Publication date: Available online 14 July 2017 Source:Stem Cell Reports Author(s): Pratibha Tripathi, Natalia Rodriguez-Muela, Joseph R. Klim, A. Sophie de Boer, Sahil Agrawal, Jackson Sandoe, Claudia S. Lopes, Karolyn Sassi Ogliari, Luis A. Williams, Matthew Shear, Lee L. Rubin, Kevin Eggan, Qiao Zhou Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease. Astrocytic factors are known to contribute to motor neuron degeneration and death in ALS. However, the role of astrocyte in promoting motor neuron protein aggregation, a disease hallmark of ALS, remains largely unclear. Here, using...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Contributors : Claire Hall ; Zhi Yao ; Minee Choi ; Giulia Tyzack ; Andrea Serio ; Raphaelle Luisier ; Jasmine Harley ; Elisavet Preza ; Charlie Arber ; Sarah J Crisp ; P M Watson ; Dimitri M Kullmann ; Andrey Abramov ; Selina Wray ; Russell Burley ; Sam H Loh ; Miguel L Martins ; Molly M Stevens ; Nicholas Luscombe ; Christopher Sibley ; Andras Lakatos ; Jernej Ule ; Sonia Gandhi ; Rickie PataniSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensMotor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interacti...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
Contributors : Claire Hall ; Zhi Yao ; Minee Choi ; Giulia Tyzack ; Andrea Serio ; Raphaelle Luisier ; Jasmine Harley ; Elisavet Preza ; Charlie Arber ; Sarah J Crisp ; P M Watson ; Dimitri M Kullmann ; Andrey Abramov ; Selina Wray ; Russell Burley ; Sam H Loh ; Miguel L Martins ; Molly M Stevens ; Nicholas Luscombe ; Christopher Sibley ; Andras Lakatos ; Jernej Ule ; Sonia Gandhi ; Rickie PataniSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensMotor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interacti...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
Amyotrophic lateral sclerosis (ALS), a fatal disease causing progressive loss of motor neurons, still has no effective treatment. We developed a phenotypic screen to repurpose existing drugs using ALS motor neuron survival as readout. Motor neurons were generated from induced pluripotent stem cells (iPSCs) derived from an ALS patient with a mutation in superoxide dismutase 1 (SOD1). Results of the screen showed that more than half of the hits targeted the Src/c-Abl signaling pathway. Src/c-Abl inhibitors increased survival of ALS iPSC-derived motor neurons in vitro. Knockdown of Src or c-Abl with small interfering RNAs (si...
Source: Science Translational Medicine - Category: Biomedical Science Authors: Tags: Reports Source Type: research
Conclusions:Identification of the mechanisms of neuronal differential vulnerability may lead to development of new therapeutic strategies that prevent the progressive neurodegeneration in MN diseases.Study Supported by: Cariplo Foundation to SC (2012-0513), Thierry Latran Foundation to SC and EH, JPND; 529-2014-7500 to SC and EH; ARISLA to SC.Disclosure: Dr. Nizzardo has nothing to disclose. Dr. Rizzo has nothing to disclose. Dr. Taiana has nothing to disclose. Dr. Allodi has nothing to disclose. Dr. Aguila Benitez has nothing to disclose. Dr. Nijssen has nothing to disclose. Dr. Ulzi has nothing to disclose. Dr. Melzi has...
Source: Neurology - Category: Neurology Authors: Tags: Neuromuscular and Clinical Neurophysiology (EMG): Motor Neuron Disease I Source Type: research
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