Marinesco-Sjögren Syndrome Protein SIL1 Regulates Motor Neuron Subtype-Selective ER Stress in ALS Scientists show that the molecular environment of the endoplasmic reticulum (ER) between motor neuron subtypes is distinct, with characteristic signatures. They identified cochaperone SIL1, mutated in Marinesco-Sjögren syndrome, as being robustly expressed in disease-resistant slow motor neurons but not in ER stress-prone fast-fatigable motor neurons. [Nat Neurosci] Abstract Targeting ATM Ameliorates Mutant Huntingtin Toxicity in Cell and Animal Models of Huntington’s Disease Researchers show that ataxia-telangiectasia mutated (ATM) signaling was consistently elevated in cells derived from Huntington’s disease (HD) mice and in brain tissue from HD mice and patients. ATM knockdown protected from toxicities induced by mutant Huntingtin fragments in mammalian cells and in transgenic Drosophila models. [Sci Transl Med] Abstract Differential Effects of Extracellular Vesicles Secreted by Mesenchymal Stem Cells from Different Sources on Glioblastoma Cells Given the ability of mesenchymal stem cells (MSCs) to affect glioma growth, researchers investigated the effects of extracellular vesicles derived from MSCs on U87MG glioblastoma cells line. [Expert Opin Biol Ther] Abstract Human Cytomegalovirus Gene Expression in Long-Term Infected Glioma Stem Cells Researchers hypothesized that a specific human cytomegalovirus (HCMV) gene signature may be associated with glioblastoma pathogenesis. They used glioma cell lines and primary glioma stem-like cells infected with clinical and laboratory HCMV strains and measured relative viral gene expression levels along several time points up to 15 weeks post-infection. [PLoS One] Full Article The Neuron Regrowth Is Associated with the Proliferation of Neural Precursor Cells after Leukemia Inhibitory Factor Administration following Spinal Cord Injury in Mice Investigators explored whether LIF could promote the proliferation of neural precursor cells (NPCs) and analyzed the correlation between increased NPCs and FluoroGold labeled neurons in mice after spinal cord injury. [PLoS One] Full Article Electrospun Biomaterial Scaffolds with Varied Topographies for Neuronal Differentiation of Human-Induced Pluripotent Stem Cells Researchers investigated the effect of micro and nanoscale scaffold topography on promoting neuronal differentiation of human induced pluripotent stem cells and directing the resulting neuronal outgrowth in an organized manner. [J Biomed Mater Res A] Abstract Identification of a Novel Cell-Penetrating Peptide Targeting Human Glioblastoma Cell Lines as a Cancer-Homing Transporter Scientists attempted to identify novel cancer-homing cell-penetrating peptides to target glioblastoma multiforme, which is often refractory and resistant to treatment. One of the candidate peptides showed selective cell-penetrating activity in U87MG cells. [Biochem Biophys Res Commun] Abstract Restoration of Progranulin Expression Rescues Cortical Neuron Generation in an Induced Pluripotent Stem Cell Model of Frontotemporal Dementia To understand how haploinsufficiency of progranulin causes frontotemporal dementia (FTD), scientists created induced pluripotent stem cells from patients carrying the GRNIVS1+5G > C mutation. Although generation of neuroprogenitors was unaffected, their further differentiation into CTIP2-, FOXP2-, or TBR1-TUJ1 double-positive cortical neurons, but not motorneurons, was significantly decreased in FTD-neural progeny. [Stem Cell Rep] Full Article | Graphical Abstract | Press Release |