Volume 5.00 | Jan 5

Neural Cell News 5.00, January 5, 2011.
In this issue:  Science News |  Current Publications |  Industry News |  Policy News |  Events
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Malfunctioning Gene Associated With Lou Gehrig’s Disease Leads to Nerve-Cell Death in Mice ShareThis
Lou Gehrig’s disease and frontotemporal lobar degeneration are characterized by protein clumps in brain and spinal-cord cells that include an RNA-binding protein called TDP-43. This protein is the major building block of the lesions formed by these clumps. Researchers describe the first direct evidence of how mutated TDP-43 can cause neurons to die. [Press release from the University of Pennsylvania School of Medicine discussing online prepublication in the Journal of Clinical Investigation]



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Neuronal Migration Errors: Right Cells, Wrong Place
Using a specialized technique that involves staining cells, the researchers were able to determine the distribution of nerve cells in brain tissue from people who had been diagnosed with schizophrenia in comparison to tissue from people who did not carry this diagnosis prior to their death. [Press release from PhysOrg discussing online prepublication in Biological Psychiatry]

Most Common Adult Brain Cancer Linked to Gene Deletion, Doctors Say
A study has identified an important gene deletion in up to one of every four cases of glioblastoma. This deletion contributes to tumor development, promotes resistance to therapy and considerably worsens a patient’s survival prospects. [Press release from the Stanford University School of Medicine discussing online prepublication in the New England Journal of Medicine]

UT Southwestern Researchers Identify Site in Brain Where Leptin may Trigger Puberty
Researchers have pinpointed a tiny site in the brain where the hormone leptin may help trigger the onset of puberty. The findings in mice indicate that a site within the hypothalamus called the ventral premammillary nucleus, or PMV, is the target where the hormone leptin effectively kick starts puberty in females. [Press release from the UT Southwestern Medical Center discussing online prepublication in the Journal of Clinical Investigation]

Brain Gene a Trigger for Determining Gender
University of Adelaide researchers are a step closer to unravelling the mysteries of human sexual development, following genetic studies that show male mice can be created without a Y chromosome – through the activation of an ancient brain gene. [Press release from the University of Adelaide discussing online prepublication in the Journal of Clinical Investigation]

Researchers Find Gene That Protects Against Dementia in High-risk Individuals
Neuroscientists had assumed that a mutation in the progranulin gene, which makes the progranulin protein and supports brain neurons, was sufficient to produce a kind of dementia known as frontotemporal lobar degeneration. But now an international team of scientists have found another genetic factor they say appears to protect against the disorder in progranulin mutation carriers. [Press release from the Mayo Clinic discussing online prepublication in Neurology]


CURRENT PUBLICATIONS (Ranked by Impact Factor of the Journal)

NFKBIA Deletion in Glioblastomas
Deletion of NFKBIA has an effect that is similar to the effect of EGFR amplification in the pathogenesis of glioblastoma and is associated with comparatively short survival. [N Engl J Med]

Leptin’s Effect on Puberty in Mice is Relayed by the Ventral Premammillary Nucleus and does not Require Signaling in Kiss1 Neurons
Here, researchers found that genetic deletion of LepR selectively from hypothalamic Kiss1 neurons in mice had no effect on puberty or fertility, indicating that direct leptin signaling in Kiss1 neurons is not required for these processes. [J Clin Invest]

Identification of SOX3 as an XX Male Sex Reversal Gene in Mice and Humans
Here, researchers report that in one of these transgenic lines, Sox3 was ectopically expressed in the bipotential gonad and that this led to frequent complete XX male sex reversal. [J Clin Invest]

Dysregulation of the ALS-Associated Gene TDP-43 Leads to Neuronal Death and Degeneration in Mice
Here, researchers generated transgenic mice expressing human TDP-43 with a defective nuclear localization signal in the forebrain (hTDP-43-deltaNLS), and compared them with mice expressing WT hTDP-43 (hTDP-43-WT) to determine the effects of mislocalized cytoplasmic TDP-43 on neuronal viability. [J Clin Invest]

Increased Interstitial White Matter Neuron Density in the Dorsolateral Prefrontal Cortex of People with Schizophrenia
Interstitial white matter neurons (IWMNs) may reflect immature neurons that migrate tangentially to the neocortex from the ganglionic eminence to form cortical interneurons. Alterations of interneuron markers have been detected in gray matter of dorsolateral prefrontal cortex in schizophrenia, and IWMNs are also reported to be altered in schizophrenia. In this study, researchers considered whether a potential link exists between these two pathological findings. [Biol Psychiat]
TMEM106B Regulates Progranulin Levels and the Penetrance of FTLD in GRN Mutation Carriers
The objective of this study was to determine whether TMEM106B single nucleotide polymorphisms are associated with frontotemporal lobar degeneration (FTLD) in patients with and without mutations in progranulin (GRN) and to determine whether TMEM106B modulates GRN expression. [Neurology]

Wnt Signaling Regulates Symmetry of Division of Neural Stem Cells in the Adult Brain and in Response to Injury
Using in vivo and in vitro models, researchers demonstrate that Wnt signaling plays a role in regulating the symmetric divisions of adult neural stem cells with no change in the proliferation kinetics of the progenitor population. [Stem Cells]

The Transfection of Multipotent Neural Precursor/Stem Cell Transplant Populations with Magnetic Nanoparticles
Researchers have evaluated the potential of magnetic nanoparticle vectors for gene transfer to neural precursor/stem cells using a neurosphere culture model system; researchers also assessed repeat transfection (“multifection”) and repeat transfection plus applied magnetic field (“magneto-multifection”) strategies [to enhance transfection efficiency]. [Biomaterials]

Grafted Human Embryonic Progenitors Expressing Neurogenin-2 Stimulate Axonal Sprouting and Improve Motor Recovery after Severe Spinal Cord Injury
Researchers investigated functional and structural outcomes after grafting human embryonic neural progenitors in spinal cord-lesioned rats. [PLoS ONE]


Biogen Idec and Neurimmune Announce Agreement on Three Neurodegenerative Disease Programs
Biogen Idec and Neurimmune Holding AG announced that Biogen Idec has acquired a subsidiary of Neurimmune, which includes the world-wide rights to three pre-clinical immunotherapy programs. [Biogen Idec Press Release]

FDA and Allon Agree on Special Protocol Assessment for Pivotal Trial in Progressive Supranuclear Palsy
Allon Therapeutics Inc. announced that it has reached agreement with the United States Food and Drug Administration (FDA) on a Special Protocol Assessment for a pivotal Phase 2/3 clinical trial to evaluate the Company’s lead neuroprotective drug candidate, davunetide, as a potential treatment for progressive supranuclear palsy, a rapidly-progressing and fatal degenerative brain disease. [Allon Therapeutics Inc. Press Release]

Aldagen Announces FDA Clearance of IND to Begin Phase 2 Study in Ischemic Stroke With Regenerative Cell Therapy
Aldagen, Inc. announced that the U.S. Food and Drug Administration (FDA) has cleared an Investigational New Drug (IND) application to conduct a clinical trial using Aldagen’s ALD-401, a unique stem cell population, for the treatment of stroke patients. [EarthTimes]

Suven Life Sciences Secures 2 Product Patents For Their NCEs
Suven Life Sciences Ltd. announced that the grant of two product patents 241484 & 241819 from Indian Patent office corresponding to the New Chemical Entities (NCEs) for the treatment of disorders associated with Neurodegenerative diseases and these Patents are valid until 2027. [Suven Life Sciences Ltd. Press Release]

Oxford BioMedica Announces Update on ProSavin® Phase I/II Study in Parkinson’s Disease
Oxford BioMedica plc, a leading gene therapy company, announces new data from the on-going Phase I/II trial of ProSavin® for the treatment of Parkinson’s disease. [Oxford Biomedica Press Release]

Researchers: Pay More Attention to Epilepsy
Epilepsy, a common and serious neurologic disorder that affects millions of people, is not getting the public attention and funding for research it deserves, according to an editorial on a study published in the January 4, 2011, print issue of Neurology, the medical journal of the American Academy of Neurology. [PhysOrg]


National Institutes of Health (United States)

Food and Drug Administration (United States)

Center for Biologics Evaluation and Research (United States)

European Medicines Agency (European Union)

Medicines and Healthcare Products Regulatory Agency (United Kingdom)

Therapeutic Goods Administration (Australia)


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