A human cellular model of amyotrophic lateral sclerosis

Nature Medicine 24, 256 (2018). doi:10.1038/nm.4509 Authors: Rebecca M Marton &Sergiu P Paşca Stem cell–derived human motor neurons were used to investigate the cellular mechanisms underlying C9ORF72-related amyotrophic lateral sclerosis.
Source: Nature Medicine - Category: General Medicine Authors: Tags: News and Views Source Type: research

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Publication date: Available online 22 April 2018Source: Journal of Clinical Orthopaedics and TraumaAuthor(s): Manoj Kumar, Sanjeev Bhoi, Keshava SharmaAbstractHematopoietic failure (HF) has been observed in trauma hemorrhagic shock (T/HS) patients. Multiple factors are involved. Elevated serum levels of cytokines, catecholamine, granulocyte colony stimulating factor, peripheral blood hematopoietic progenitor cells (HPCs) and decreased expression of erythropoietin receptor are associated with HF among T/HS. HF leads to anaemia, susceptibility to infection, sepsis and multi-organ failure. There is a lack of molecular underst...
Source: Journal of Clinical Orthopaedics and Trauma - Category: Orthopaedics Source Type: research
Publication date: 5 June 2018Source: Stem Cell Reports, Volume 10, Issue 6Author(s): Aslam Abbasi Akhtar, Genevieve Gowing, Naomi Kobritz, Steve E. Savinoff, Leslie Garcia, David Saxon, Noell Cho, Gibum Kim, Colton M. Tom, Hannah Park, George Lawless, Brandon C. Shelley, Virginia B. Mattis, Joshua J. Breunig, Clive N. SvendsenSummaryTrophic factor delivery to the brain using stem cell-derived neural progenitors is a powerful way to bypass the blood-brain barrier. Protection of diseased neurons using this technology is a promising therapy for neurodegenerative diseases. Glial cell line-derived neurotrophic factor (GDNF) has...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: 5 June 2018Source: Stem Cell Reports, Volume 10, Issue 6Author(s): Evangelos Kiskinis, Joel M. Kralj, Peng Zou, Eli N. Weinstein, Hongkang Zhang, Konstantinos Tsioras, Ole Wiskow, J. Alberto Ortega, Kevin Eggan, Adam E. CohenSummaryHuman induced pluripotent stem cell (iPSC)-derived neurons are an attractive substrate for modeling disease, yet the heterogeneity of these cultures presents a challenge for functional characterization by manual patch-clamp electrophysiology. Here, we describe an optimized all-optical electrophysiology, “Optopatch,” pipeline for high-throughput functional characteri...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Julia Higelin, Alberto Catanese, Lena Luisa Semelink-Sedlacek, Sertap Oeztuerk, Anne-Kathrin Lutz, Julia Bausinger, Gotthold Barbi, Günter Speit, Peter M. Andersen, Albert C. Ludolph, Maria Demestre, Tobias M. BoeckersAbstractMutations in genes coding for proteins involved in DNA damage response (DDR) and repair, such as C9orf72 and FUS (Fused in Sarcoma), are associated with neurodegenerative diseases and lead to amyotrophic lateral sclerosis (ALS). Heterozygous loss-of-function mutations in NEK1 (NIMA-related kinase 1) have also been recently...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
Publication date: Available online 22 April 2018Source: Journal of Clinical Orthopaedics and TraumaAuthor(s): Manoj Kumar, Sanjeev Bhoi, Keshava SharmaAbstractHematopoietic failure (HF) has been observed in trauma hemorrhagic shock (T/HS) patients. Multiple factors are involved. Elevated serum levels of cytokines, catecholamine, granulocyte colony stimulating factor, peripheral blood hematopoietic progenitor cells (HPCs) and decreased expression of erythropoietin receptor are associated with HF among T/HS. HF leads to anaemia, susceptibility to infection, sepsis and multi-organ failure. There is a lack of molecular underst...
Source: Journal of Clinical Orthopaedics and Trauma - Category: Orthopaedics Source Type: research
Publication date: 15 August 2018Source: Molecular and Cellular Endocrinology, Volume 471Author(s): Zoltan Simandi, Attila Horvath, Ixchelt Cuaranta-Monroy, Sascha Sauer, Jean-Francois Deleuze, Laszlo NagyAbstractRetinoid X Receptors (RXRs) are unique and enigmatic members of the nuclear receptor (NR) family with extensive and complex biological functions in cellular differentiation. On the one hand, RXRs through permissive heterodimerization with other NRs are able to integrate multiple lipid signaling pathways and are believed to play a central role to coordinate the development of the central nervous system. On the other...
Source: Molecular and Cellular Endocrinology - Category: Endocrinology Source Type: research
Publication date: 5 June 2018Source: Stem Cell Reports, Volume 10, Issue 6Author(s): Aslam Abbasi Akhtar, Genevieve Gowing, Naomi Kobritz, Steve E. Savinoff, Leslie Garcia, David Saxon, Noell Cho, Gibum Kim, Colton M. Tom, Hannah Park, George Lawless, Brandon C. Shelley, Virginia B. Mattis, Joshua J. Breunig, Clive N. SvendsenSummaryTrophic factor delivery to the brain using stem cell-derived neural progenitors is a powerful way to bypass the blood-brain barrier. Protection of diseased neurons using this technology is a promising therapy for neurodegenerative diseases. Glial cell line-derived neurotrophic factor (GDNF) has...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: 5 June 2018Source: Stem Cell Reports, Volume 10, Issue 6Author(s): Evangelos Kiskinis, Joel M. Kralj, Peng Zou, Eli N. Weinstein, Hongkang Zhang, Konstantinos Tsioras, Ole Wiskow, J. Alberto Ortega, Kevin Eggan, Adam E. CohenSummaryHuman induced pluripotent stem cell (iPSC)-derived neurons are an attractive substrate for modeling disease, yet the heterogeneity of these cultures presents a challenge for functional characterization by manual patch-clamp electrophysiology. Here, we describe an optimized all-optical electrophysiology, “Optopatch,” pipeline for high-throughput functional characteri...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: July 2018Source: Stem Cell Research, Volume 30Author(s): Julia Higelin, Alberto Catanese, Lena Luisa Semelink-Sedlacek, Sertap Oeztuerk, Anne-Kathrin Lutz, Julia Bausinger, Gotthold Barbi, Günter Speit, Peter M. Andersen, Albert C. Ludolph, Maria Demestre, Tobias M. BoeckersAbstractMutations in genes coding for proteins involved in DNA damage response (DDR) and repair, such as C9orf72 and FUS (Fused in Sarcoma), are associated with neurodegenerative diseases and lead to amyotrophic lateral sclerosis (ALS). Heterozygous loss-of-function mutations in NEK1 (NIMA-related kinase 1) have also been recently...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
AbstractSporadic amyotrophic lateral sclerosis (sALS) is the most common form of ALS, however, the molecular mechanisms underlying cellular damage and motor neuron degeneration remain elusive. To identify molecular signatures of sALS we performed genome-wide expression profiling in laser capture microdissection-enriched surviving motor neurons (MNs) from lumbar spinal cords of sALS patients with rostral onset and caudal progression. After correcting for immunological background, we discover a highly specific gene expression signature for sALS that is associated with phosphorylated TDP-43 (pTDP-43) pathology. Transcriptome ...
Source: Acta Neuropathologica - Category: Neurology Source Type: research
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