Neural Cell News Volume 8.23 | Jun 18 2014

TorsinA Hypofunction Causes Abnormal Twisting Movements and Sensorimotor Circuit Neurodegeneration
Scientists found that conditional deletion of Tor1a in the CNS (nestin-Cre Tor1a^flox/–) or isolated CNS expression of DYT1 mutant torsinA (nestin-Cre Tor1a^flox/ΔE) causes striking abnormal twisting movements. These animals developed perinuclear accumulation of ubiquitin and the E3 ubiquitin ligase HRD1 in discrete sensorimotor regions, followed by neurodegeneration that was substantially milder in nestin-Cre Tor1a^flox/ΔE compared with nestin-Cre Tor1a^flox/– animals. [J Clin Invest] Full Article | Press Release

Stromal Heparan Sulfate Differentiates Neuroblasts to Suppress Neuroblastoma Growth
Investigators identified critical growth-limiting components of the differentiating stroma secretome and designed a potential therapeutic strategy based on their central mechanism of action. They demonstrated that expression of heparan sulfate proteoglycans, including TβRIII, GPC1, GPC3, SDC3, and SDC4, is low in neuroblasts and high in the Schwannian stroma. [J Clin Invest] Full Article | Press Release

Increased Vesicular Monoamine Transporter Enhances Dopamine Release and Opposes Parkinson Disease-Related Neurodegeneration In Vivo
Researchers report a novel mouse model of enhanced vesicular function via bacterial artificial chromosome-mediated overexpression of the vesicular monoamine transporter 2. These mice exhibit significant protection from neurotoxic insult by the dopaminergic toxicant 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, as measured by reduced dopamine terminal damage and substantia nigra pars compacta cell loss. [Proc Natl Acad Sci USA] Abstract | Press Release

Sparse and Distributed Coding of Episodic Memory in Neurons of the Human Hippocampus
The authors investigated the representation of episodic memory in hippocampal neurons of nine epilepsy patients undergoing intracranial monitoring as they discriminated between recently studied words (targets) and new words (foils) on a recognition test. [Proc Natl Acad Sci USA] Abstract | Press Release

Improvement of the Rett Syndrome Phenotype in a Mecp2 Mouse Model Upon Treatment with Levodopa and a Dopa Decarboxylase Inhibitor
Investigators showed that the combined administration of Levodopa and a Dopa decarboxylase inhibitor in Rett syndrome mouse models is well tolerated, diminishes Rett syndrome-associated symptoms and increases lifespan. Most important, the improvement of the Rett syndrome phenotype upon use of the combined treatment is reflected at the cellular level by the development of neuronal dendritic growth. [Neuropsychopharmacology] Abstract | Full Article | Press Release

Fluorescent Protein-Expressing Neural Progenitor Cells as a Tool for Transplantation Studies
Using immunocytochemistry, immunoblotting, flow cytometry and electrophysiology, quadruple transgenic neural progenitor cells (NPCs) and green fluorescent protein-sorted NPCs were comprehensively characterized in vitro. [PLoS One] Full Article

α-Synuclein Immunotherapy Blocks Uptake and Templated Propagation of Misfolded α-Synuclein and Neurodegeneration
Scientists demonstrated that alpha-synuclein (α-syn) monoclonal antibodies reduce α-syn preformed fibril (pff)-induced Lewy body/Lewy neurite formation and rescue synapse/neuron loss in primary neuronal cultures by preventing both pff uptake and subsequent cell-to-cell transmission of pathology. [Cell Reports]
Full Article | Graphical Abstract | Press Release

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FDA Grants Fast Track Status to DNAtrix Drug DNX-2401 for Recurrent Glioblastoma
DNAtrix, Inc. announced that the U.S. Food and Drug Administration (FDA) has granted Fast Track status for its lead product, DNX-2401, a replication competent adenovirus, for patients with recurrent gliobastoma. [DNAtrix, Inc.] Press Release

CCRM and Stem Cells Australia Agree to Collaborate in the Global Translation of Stem Cell Research
The Centre for Commercialization of Regenerative Medicine (CCRM) and the Stem Cells Australia consortium have entered into a memorandum of understanding to work together to increase the translation of stem cell research in the two countries. [Stem Cells Australia] Press Release

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