Neural Cell News Volume 9.34 | Sep 9 2015

The C9orf72 Repeat Expansion Disrupts Nucleocytoplasmic Transport
RanGAP physically interacts with hexanucleotide repeat expansion (HRE) RNA and is mislocalized in HRE-expressing flies, neurons from C9orf72 amyotrophic lateral sclerosis (ALS) patient-derived induced pluripotent stem cells, and in C9orf72 ALS patient brain tissue. [Nature] Abstract

Modifiers of C9orf72 Dipeptide Repeat Toxicity Connect Nucleocytoplasmic Transport Defects to FTD/ALS
Dipeptide repeat proteins produced by unconventional translation of the C9orf72 repeat expansions caused neurodegeneration in cell culture and in animal models. [Nat Neurosci] Abstract | Press Release

Altered Neuronal Network and Rescue in a Human MECP2 Duplication Model
The authors generated induced pluripotent stem cells from patients with the methyl-CpG-binding protein-2 (MECP2) duplication syndrome, carrying different duplication sizes, to study the impact of increased MeCP2 dosage in human neurons. [Mol Psych] Abstract | Press Release

The Myelin Oligodendrocyte Glycoprotein Directly Binds Nerve Growth Factor to Modulate Central Axon Circuitry
The authors identified nerve growth factor (NGF) as a binding partner for myelin oligodendrocyte glycoprotein and demonstrated that this interaction is capable of sequestering NGF from TrkA-expressing neurons to modulate axon growth and survival. [J Cell Biol] Abstract

Transcription Factors Sox5 and Sox6 Exert Direct and Indirect Influences on Oligodendroglial Migration in Spinal Cord and Forebrain
Using mice with specific deletion of the SoxD proteins Sox5 and Sox6 in the central nervous system, scientists showed that SoxD proteins additionally influence migration of oligodendrocyte progenitors in the spinal cord as well as in the forebrain. [Glia] Abstract

A Septo-Temporal Molecular Gradient of sfrp3 in the Dentate Gyrus Differentially Regulates Quiescent Adult Hippocampal Neural Stem Cell Activation
Scientists showed that activation rates within the quiescent radial glia-like neural stem cell (RGL) population decrease gradually along the septo-temporal axis in the adult mouse dentate gyrus, as defined by MCM2 expression in RGLs. [Mol Brain] Full Article

Subventricular Zone Neural Progenitors Reverse TNF-Alpha Effects in Cortical Neurons
Investigators demonstrated that adult neural stem cells (aNPCs) from the subventricular zone reverse the effects induced by the cytokine. Moreover, they showed that the effect of aNPCs on cortical neurons is mediated by cannabinoid CB1 receptor activation. [Stem Cell Res Ther] Full Article

Agmatine Protects Against 6-OHDA-Induced Apoptosis, and ERK and Akt/GSK Disruption in SH-SY5Y Cells
Scientists assessed whether agmatine prevents 6-hydroxydopamine (6-OHDA)-induced SH-SY5Y cell death and if so, then how it affects Akt/glycogen synthesis kinase(GSK)-3β and extracellular signal-regulated kinases (ERK) signals. [Cell Mol Neurobiol] Abstract

Indirubin-3-Oxime Effectively Prevents 6OHDA-Induced Neurotoxicity in PC12 Cells via Activating MEF2D through the Inhibition of GSK3β
Researchers showed that indirubin-3-oxime prevented 6-hydroxydopamine (6OHDA)-induced neuronal apoptosis and intracellular reactive oxygen species accumulation in PC12 cells in a concentration-dependent manner. [J Mol Neurosci] Abstract

Development of Stem Cell-Based Therapy for Parkinson’s Disease
The authors evaluate the research progress in different cell sources with a focus on using induced pluripotent stem cells as a valuable source and propose key challenges for developing cells suitable for large-scale clinical applications in the treatment of Parkinson’s disease. [Transl Neurodegener] Full Article

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Johns Hopkins University, Cedars-Sinai and Massachusetts General Hospital Announce Groundbreaking ALS Research Project of Answer ALS
Johns Hopkins University’s Robert Packard Center for ALS Research, Cedars-Sinai Medical Center’s Regenerative Medicine Institute and the Massachusetts General Hospital Neurological Clinical Research Institute announced the launch of an Amyotrophic Lateral Sclerosis (ALS) research project of Answer ALS, the largest single coordinated and comprehensive effort to end ALS. [Johns Hopkins University (Business Wire)] Press Release

Celsion Completes Integration of EGEN and Outlines Clinical Development Plan for GEN-1 IL-12 Immunotherapy Program
Celsion Corporation announced that the company has completed the integration of its June 2014 acquisition of EGEN, Inc. and has provided an update on its clinical development plans for GEN-1, the company’s DNA-based immunotherapy for the localized treatment of ovarian and brain cancer. [Celsion Corporation] Press Release

Mayo Clinic and Hootsuite Introduce Social Media Training Program for Health Care
Hootsuite and Mayo Clinic Center for Social Media, announced an industry-leading social media credential for medical and health care professionals. [Hootsuite Media Inc.] Press Release

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