Neural Cell News Volume 7.21 | May 29 2013

Exome Sequencing to Identify De Novo Mutations in Sporadic ALS Trios
To identify additional genetic risk factors, investigators assessed the role of de novo mutations in amyotrophic lateral sclerosis (ALS) by sequencing the exomes of 47 ALS patients and both of their unaffected parents. CREST mutations inhibited activity-dependent neurite outgrowth in primary neurons, and CREST associated with the ALS protein FUS. [Nat Neurosci] Abstract | Press Release

Diet Controls Drosophila Follicle Stem Cell Proliferation via Hedgehog Sequestration and Release
Researchers elucidated the mechanism by which dietary cholesterol controls epithelial follicle stem cell (FSC) proliferation in the fly ovary. In nutrient-restricted flies, the transmembrane protein Boi sequesters Hedgehog (Hh) ligand at the surface of Hh-producing cells within the ovary, limiting FSC proliferation. [J Cell Biol] Abstract

Deficits in Human Trisomy 21 iPSCs and Neurons
Researchers created trisomy of chromosome 21 (Ts21)-induced pluripotent stem cells (iPSCs) from two sets of Ts21 human fibroblasts. Differentiation of all Ts21 iPSCs yielded similar numbers of neurons expressing markers characteristic of dorsal forebrain neurons that were functionally similar to controls. [Proc Natl Acad Sci USA] Abstract | Full Article | Press Release

Expression of ALS-Linked TDP-43 Mutant in Astrocytes Causes Non-Cell-Autonomous Motor Neuron Death in Rats
The authors created novel lines of transgenic rats that express a mutant form of human Tar DNA-binding protein 43 (TDP-43) restricted to astrocytes. Selective expression of mutant TDP-43 in astrocytes caused a progressive loss of motor neurons and the denervation atrophy of skeletal muscles, resulting in progressive paralysis. [EMBO J] Abstract

The Functional Genetic Link of NLGN4X Knockdown and Neurodevelopment in Neural Stem Cells
Researchers used neural stem cells (NSCs) as in vitro models to explore the impact of NLGN4X knockdown on neurodevelopment. At the cellular level, in NSCs with NLGN4X knockdown, they observed increasingly delayed neuronal development and compromised neurite formation, starting from week two through week six post differentiation. [Hum Mol Gen] Abstract

Reduced Adult Hippocampal Neurogenesis and Working Memory Deficits in the Dgcr8-Deficient Mouse Model of 22q11.2 Deletion-Associated Schizophrenia Can Be Rescued by IGF2
Scientists showed that DiGeorge syndrome chromosomal region 8 (Dgcr8) heterozygosity in mice leads to reduced cell proliferation and neurogenesis in adult hippocampus, as well as impaired hippocampus-dependent learning. Several schizophrenia-associated genes were downregulated in the hippocampus of Dgcr8^+/− mice, and one of them, insulin-like growth factor 2, rescued the proliferation of adult neural stem cells both in vitro and in vivo. [J Neurosci] Abstract

Synergistic Effects of GDNF and VEGF on Lifespan and Disease Progression in a Familial ALS Rat Model
Scientists have recently shown that human mesenchymal stem cells modified to release glial cell line-derived neurotrophic factor (GDNF) decrease disease progression in a rat model of amyotrophic lateral sclerosis (ALS) when delivered to skeletal muscle. In the current study, they determined whether or not this effect could be enhanced by delivering GDNF in concert with other trophic factors. [Mol Ther] Abstract | Press Release

Functional Astrocyte-Neuron Lactate Shuttle in a Human Stem Cell-Derived Neuronal Network
Researchers showed that human stem cell-derived astrocytes produce glycogen and that co-cultures of these cells demonstrate a functional astrocyte-neuron lactate shuttle (ANLS). The ANLS hypothesis proposes that during neuronal activity, glutamate released into the synaptic cleft is taken up by astrocytes and triggers glucose uptake, which is converted into lactate and released via monocarboxylate transporters for neuronal use. [J Cerebr Blood F Met] Abstract

Restoration of Sensitivity in Chemo – Resistant Glioma Cells by Cold Atmospheric Plasma
Scientists demonstrated the anti-cancer properties of different dosages of cold atmospheric plasma (CAP) both in temozolomide (TMZ)-sensitive and TMZ-resistant cells by proliferation assay, immunoblotting, cell cycle analysis, and clonogenicity assay. Importantly, CAP treatment restored the responsiveness of resistant glioma cells towards TMZ therapy. [PLoS One] Full Article | Press Release

Amelioration of Motor/Sensory Dysfunction and Spasticity in a Rat Model of Acute Lumbar Spinal Cord Injury by Human Neural Stem Cell Transplantation
Scientists characterized the effect of intraspinal grafting of clinical grade human fetal spinal cord-derived neural stem cells on the recovery of neurological function in a rat model of acute lumbar compression injury. [Stem Cell Res Ther] Abstract | Full Article | Press Release

Neuroblastoma: Developmental Biology, Cancer Genomics and Immunotherapy
In this review the authors discuss the key discoveries in the developmental biology, molecular genetics and immunology of neuroblastoma, as well as new translational tools for bringing these promising scientific advances into the clinic. [Nat Rev Cancer] Abstract

Visit our reviews page to see a complete list of reviews in the neuroscience field.

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AxoGen, Inc. Receives Grant in Partnership with Vanderbilt University from U.S. Department of Defense
AxoGen, Inc. announced that the Company and Vanderbilt University have been awarded a shared $1.1 million grant, for which AxoGen’s portion is approximately 35%, from the Congressional Directed Medical Research Programs administrated by the U.S. Department of Defense to support the development and expansion of nerve repair technologies. [AxoGen, Inc.] Press Release

Merck Provides Update on Phase III Clinical Program for Preladenant, the Company’s Investigational Parkinson’s Disease Medicine
Merck provided an update on the clinical program for preladenant, Merck’s investigational adenosine A2A receptor antagonist for the treatment of Parkinson’s disease. An initial review of data from three separate Phase III trials did not provide evidence of efficacy for preladenant compared with placebo. [Merck & Co., Inc.] Press Release

Agenus Announces Initiation of Enrollment in National Cancer Institute-Sponsored Randomized Trial of Prophage G-200 Vaccine with Avastin in Treatment of Brain Tumors
Agenus Inc. announced enrollment has started for a large, randomized Phase II study of Prophage G-200 (Heat Shock Protein Peptide Complex-96 or HSPPC-96) vaccine in combination with bevacizumab (Avastin®) for treatment of recurrent glioblastoma multiforme in adult patients. [Agenus Inc.] Press Release

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NEW Postdoctoral Position – Neuronal Differentiation and Migration (Fonds de la Recherche Scientifique)

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