Disorders of the nervous system

• Presenilin mutations linked to familial Alzheimer's disease cause an imbalance in phosphatidylinositol 4,5-bisphosphate metabolism

  Landman, N. et al.
  PNAS
  (13 December 2006); doi:10.1073/pnas.0604954103

  Abstract

• MEKK4 Signaling Regulates Filamin Expression and Neuronal Migration

  Sarkisian, M. R. et al.
  Neuron 52 789-801
  (7 December 2006); doi:10.1016/j.neuron.2006.10.024

  Abstract

• ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease

  Ryu, H. et al.
  PNAS 103 19176-19181
  (1 December 2006); doi:10.1073/pnas.0606373103

  Abstract

• Seizures and enhanced cortical GABAergic inhibition in two mouse models of human autosomal dominant nocturnal frontal lobe epilepsy

  Klaassen, A. et al.
  PNAS 103 19152-19157
  (4 December 2006); doi:10.1073/pnas.0608215103

  Abstract

• GGA1 Is Expressed in the Human Brain and Affects the Generation of Amyloid β-Peptide

  Wahle, T. et al.
  The Journal of Neuroscience 26 12838-12846
  doi:10.1523/JNEUROSCI.1982-06.2006

  Abstract

• Neuronal Pentraxin 1 Contributes to the Neuronal Damage Evoked by Amyloid-β and Is Overexpressed in Dystrophic Neurites in Alzheimer's Brain

  Abad, M. A., Enguita, M., DeGregorio-Rocasolano, N., Ferrer, I. & Trullas, R.
  The Journal of Neuroscience 26
  (6 December 2006); doi:10.1523/JNEUROSCI.0575-06.2006

  Abstract

• Toll-Like Receptor 2 Signaling in Response to Brain Injury: An Innate Bridge to Neuroinflammation

  Babcock, A. A. et al.
  The Journal of Neuroscience
  (6 December 2006); doi:10.1523/JNEUROSCI.4937-05.2006

  Abstract

• A polymorphism in the norepinephrine transporter gene alters promoter activity and is associated with attention-deficit hyperactivity disorder

  Kim, C. et al.
  The National Academy of Sciences 10319164-19169
  doi:10.1073/pnas.0510836103

  Abstract

• RORα-Mediated Purkinje Cell Development Determines Disease Severity in Adult SCA1 Mice

  Serra, H. G. et al.
  Cell 127
  (17 November 2006); doi:10.1016/j.cell.2006.09.036

  Abstract

• The Familial Parkinsonism Gene LRRK2 Regulates Neurite Process Morphology

  MacLeod, D. et al.
  Neuron 52
  (22 November 2006); doi:10.1016/j.neuron.2006.10.008

  Abstract

• LRRTM3 promotes processing of amyloid-precursor protein by BACE1 and is a positional candidate gene for late-onset Alzheimer's disease

  Majercak, J. et al.
  Proceedings of the National Academy of Sciences 103
  (21 November 2006); doi:10.1073/pnas.0605461103

  Abstract

• The ducky2J Mutation in Cacna2d2 Results in Reduced Spontaneous Purkinje Cell Activity and Altered Gene Expression

  Donato, R. et al.
  Journal of Neuroscience 26
  (29 November 2006); doi:10.1523/JNEUROSCI.3080-06.2006

  Abstract

• From the Cover: Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome

  McGill, B. E. et al.
  Proceedings of the National Academy of Sciences 103
  (15 November 2006); doi:10.1073/pnas.0608702103

  Abstract

• The neuronal microRNA system

  Kenneth S. Kosik
  Nature Reviews Neuroscience 7 911 - 920
  doi:10.1038/nrn2037

  Abstract

• A neuregulin 1 variant associated with abnormal cortical function and psychotic symptoms

  Jeremy Hall, Heather C Whalley, Dominic E Job, Ben J Baig, Andrew M McIntosh, Kathryn L Evans, Pippa A Thomson, David J Porteous, David G Cunningham-Owens, Eve C Johnstone and Stephen M Lawrie
  Nature Neuroscience
  (29 October 2006); doi:10.1038/nn1795

  Abstract

• Retinal repair by transplantation of photoreceptor precursors

  R. E. MacLaren, R. A. Pearson, A. MacNeil, R. H. Douglas, T. E. Salt, M. Akimoto, A. Swaroop, J. C. Sowden and R. R. Ali
  Nature 444 203 - 207
  doi:10.1038/nature05161

  Abstract

• Expression of Mutated Mouse Myocilin Induces Open-Angle Glaucoma in Transgenic Mice

  Senatorov, V. et al.
  Journal of Neuroscience 26
  (15 November 2006); doi:10.1523/JNEUROSCI.3020-06.2006

  Abstract

• From the Cover: A genetic variant that disrupts MET transcription is associated with autism

  Campbell, D. B. et al.
  Proceedings of the National Academy of Sciences 103
  (19 October 2006); doi:10.1073/pnas.0605296103

  Abstract

• Brain-Specific Phosphorylation of MeCP2 Regulates Activity-Dependent Bdnf Transcription, Dendritic Growth, and Spine Maturation

  Zhou, Z. et al.
  Neuron 52
  (18 October 2006); doi:10.1016/j.neuron.2006.09.037

  Abstract

• Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

  Beers, D. R. et al.
  Proceedings of the National Academy of Sciences 103
  (16 October 2006); doi:10.1073/pnas.0607423103

  Abstract

• A Century of Alzheimer's Disease

  Goedert, M. & Spillantini, M. G.
  Science 314
  (03 November 2006); doi:10.1126/science.1132814

  Abstract

• 100 Years and Counting: Prospects for Defeating Alzheimer's Disease

  Roberson, E. D. & Mucke, L.
  Science 314
  (03 November 2006); doi:10.1126/science.1132813

  Abstract

• Activated protein C inhibits tissue plasminogen activator–induced brain hemorrhage

  Tong Cheng, Anthony L Petraglia, Zhang Li, Meenakshisundaram Thiyagarajan, Zhihui Zhong, Zhenhua Wu, Dong Liu, Sanjay B Maggirwar, Rashid Deane, José A Fernández, Barbra LaRue, John H Griffin, Michael Chopp and Berislav V Zlokovic
  Nature Medicine 12 1278 - 1285
  (29 October 2006); doi:10.1038/nm1498

  Abstract

• Rescuing qkv Dysmyelination by a Single Isoform of the Selective RNA-Binding Protein QKI

  Zhao, L., Tian, D., Xia, M., Macklin, W. B. & Feng, Y.
  Journal of Neuroscience 26
  (01 November 2006); doi:10.1523/JNEUROSCI.2677-06.2006

  Abstract

• Ataxin-3 Represses Transcription via Chromatin Binding, Interaction with Histone Deacetylase 3, and Histone Deacetylation

  Evert, B. O. et al.
  Journal of Neuroscience 26
  (01 November 2006); doi:10.1523/JNEUROSCI.2053-06.2006

  Abstract

• Transgenic Rescue of ataxia Mice with Neuronal-Specific Expression of Ubiquitin-Specific Protease 14

  Crimmins, S. et al.
  Journal of Neuroscience 26
  (01 November 2006); doi:10.1523/JNEUROSCI.3600-06.2006

  Abstract

• Matrix Metalloproteinases Expressed by Astrocytes Mediate Extracellular Amyloid-beta Peptide Catabolism

  Yin, K. et al.
  Journal of Neuroscience 26
  (25 October 2006); doi:10.1523/JNEUROSCI.2085-06.2006

  Abstract

• Disrupted in Schizophrenia 1 Interactome: evidence for the close connectivity of risk genes and a potential synaptic basis for schizophrenia

  L M Camargo, V Collura, J-C Rain, K Mizuguchi, H Hermjakob, S Kerrien, T P Bonnert, P J Whiting and N J Brandon
  Molecular Psychiatry
  (17 October 2006); doi:10.1038/sj.mp.4001880

  Abstract

• Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

  Neumann, M. et al.
  Science
  (6 October 2006); doi:10.1126/science.1134108

  Abstract

• The neurodegenerative disease protein aprataxin resolves abortive DNA ligation intermediates

  Ivan Ahel, Ulrich Rass, Sherif F. El-Khamisy, Sachin Katyal, Paula M. Clements, Peter J. McKinnon, Keith W. Caldecott and Stephen C. West
  Nature 443 713 - 716
  (10 September 2006); doi:10.1038/nature05164

  Abstract

• Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

  Michael T. Lin and M. Flint Beal
  Nature 443 787 - 795
  (18 October 2006); doi:10.1038/nature05292

  Abstract

• Cell death in the nervous system

  Dale E. Bredesen, Rammohan V. Rao and Patrick Mehlen
  Nature 443 796 - 802
  (18 October 2006); doi:10.1038/nature05293

  Abstract

• Dysregulation of Brain-Derived Neurotrophic Factor Expression and Neurosecretory Function in Mecp2 Null Mice

  Wang, H. et al.
  Journal of Neuroscience
  (18 October 2006); doi:10.1523/JNEUROSCI.1810-06.2006

  Abstract

• An Inhibitor of Serine Proteases, Neuroserpin, Acts as a Neuroprotective Agent in a Mouse Model of Neurodegenerative Disease

  Simonin, Y., Charron, Y., Sonderegger, P., Vassalli, J. & Kato, A. C.
  Journal of Neuroscience
  (11 October 2006); doi:10.1523/JNEUROSCI.3582-06.2006

  Abstract

• Kallmann Syndrome: Mutations in the Genes Encoding Prokineticin-2 and Prokineticin Receptor-2

  Dodé, C. et al.
  PLoS Genetics 2
  (20 October 2006) doi:10.1371/journal.pgen.0020175

  Abstract

• Lamin B1 duplications cause autosomal dominant leukodystrophy

  Quasar S Padiath, Kazumasa Saigoh, Raphael Schiffmann, Hideaki Asahara, Takeshi Yamada, Anulf Koeppen, Kirk Hogan, Louis J Ptáek and Ying-Hui Fu
  Nature Genetics 38 1114 - 1123
  (3 September 2006); doi:10.1038/ng1872

  Abstract

• A common CFH haplotype, with deletion of CFHR1 and CFHR3, is associated with lower risk of age-related macular degeneration

  Anne E Hughes, Nick Orr, Hossein Esfandiary, Martha Diaz-Torres, Timothy Goodship and Usha Chakravarthy
  Nature Genetics 38 1173 - 1177
  (24 September 2006); doi:10.1038/ng1890

  Abstract

• Control of Peripheral Nerve Myelination by the β-Secretase BACE1

  Willem, M. et al.
  Science
  (21 September 2006); doi:10.1126/science.1132341

  Abstract

• Targeted Deletion of a Single Sca8 Ataxia Locus Allele in Mice Causes Abnormal Gait, Progressive Loss of Motor Coordination, and Purkinje Cell Dendritic Deficits

  He, Y., Zu, T., Benzow, K. A., Orr, H. T., Clark, H. B. & Koob, M. D.
  Journal of Neuroscience 26
  (27 September 2006); doi:10.1523/JNEUROSCI.2595-06.2006

  Abstract

• RTP801 Is Elevated in Parkinson Brain Substantia Nigral Neurons and Mediates Death in Cellular Models of Parkinson's Disease by a Mechanism Involving Mammalian Target of Rapamycin Inactivation

  Malagelada, C., Ryu, E. J., Biswas, S. C., Jackson-Lewish, V. & Greene, L. A.
  Journal of Neuroscience 26
  (27 September 2006); doi:10.1523/JNEUROSCI.3292-06.2006

  Abstract

• Deficiency of hyccin, a newly identified membrane protein, causes hypomyelination and congenital cataract

  Federico Zara, Roberta Biancheri, Claudio Bruno, Laura Bordo, Stefania Assereto, Elisabetta Gazzerro, Federica Sotgia, Xiao Bo Wang, Stefania Gianotti, Silvia Stringara, Marina Pedemonte, Graziella Uziel, Andrea Rossi, Angelo Schenone, Paolo Tortori-Donati, Marjo S van der Knaap, Michael P Lisanti and Carlo Minetti
  Nature Genetics 38 1111 - 1113
  (3 September 2006); doi:10.1038/ng1870

  Abstract

• Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase

  Alfredo Ramirez, André Heimbach, Jan Gründemann, Barbara Stiller, Dan Hampshire, L Pablo Cid, Ingrid Goebel, Ammar F Mubaidin, Abdul-Latif Wriekat, Jochen Roeper, Amir Al-Din, Axel M Hillmer, Meliha Karsak, Birgit Liss, C Geoffrey Woods, Maria I Behrens and Christian Kubisch
  Nature Genetics 38 1184 - 1191
  (10 September 2006); doi:10.1038/ng1884

  Abstract

• Effects in Neocortical Neurons of Mutations of the Nav1.2 Na+ Channel causing Benign Familial Neonatal-Infantile Seizures

  Scalmani, P. et al.
  Journal of Neuroscience
  (4 October 2006); doi:10.1523/JNEUROSCI.2476-06.2006

  Abstract

• Expression of Phox2b by Brainstem Neurons Involved in Chemosensory Integration in the Adult Rat

  Stornetta, R. L. et al.
  Journal of Neuroscience
  (4 October 2006); doi:10.1523/JNEUROSCI.2917-06.2006

  Abstract

• Slow progressive degeneration of nigral dopaminergic neurons in postnatal Engrailed mutant mice

  Sgado, P. et al.
  Proceedings of the National Academy of Sciences
  (2 October 2006); doi:10.1073/pnas.0602116103

  Abstract

• Inaugural Article: wasted away, a Drosophila mutation in triosephosphate isomerase, causes paralysis, neurodegeneration, and early death

  Gnerer, J. P., Kreber, R. A. & Ganetzky, B.
  Proceedings of the National Academy of Sciences
  (28 September 2006); doi:10.1073/pnas.0606887103

  Abstract

• Genomic Rearrangements and Gene Copy-Number Alterations as a Cause of Nervous System Disorders

  Lee, J. A. & Lupski, J. R.
  Neuron 52
  (5 October 2006); doi:10.1016/j.neuron.2006.09.027

  Abstract

• The Neurobiology of Multiple Sclerosis: Genes, Inflammation, and Neurodegeneration

  Hauser, S. L. & Oksenberg, J. R.
  Neuron 52
  (5 October 2006); doi:10.1016/j.neuron.2006.09.011

  Abstract

• From genes to behavior in developmental dyslexia

  Albert M Galaburda, Joseph LoTurco, Franck Ramus, R Holly Fitch and Glenn D Rosen
  Nature Neuroscience 9 1213 - 1217
  (26 September 2006); doi:10.1038/nn1772

  Abstract

• Fragile X syndrome and autism at the intersection of genetic and neural networks

  Matthew K Belmonte and Thomas Bourgeron
  Nature Neuroscience 9 1221 - 1225
  (26 September 2006); doi:10.1038/nn1765

  Abstract

• Time to give up on a single explanation for autism

  Francesca Happé, Angelica Ronald and Robert Plomin
  Nature Neuroscience 9 1218 - 1220
  (26 September 2006); doi:10.1038/nn1770

  Abstract

• Presenilins Form ER Ca2+ Leak Channels, a Function Disrupted by Familial Alzheimer's Disease-Linked Mutations

  Tu, H. et al.
  Cell 126
  (8 September 2006); doi:10.1016/j.cell.2006.06.059

  Abstract

• Pregnane X receptor (PXR) activation: A mechanism for neuroprotection in a mouse model of Niemann-Pick C disease

  Langmade, S. J. et al.
  Proceedings of the National Academy of Sciences 103
  (29 August 2006); doi:10.1073/pnas.0606218103

  Abstract

• Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6

  Poët, M. et al.
  Proceedings of the National Academy of Sciences 103
  (1 September 2006); doi:10.1073/pnas.0606137103

  Abstract

• Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials

  Ferri, A. et al.
  Proceedings of the National Academy of Sciences 103
  (30 August 2006); doi:10.1073/pnas.0605814103

  Abstract

• α-Synuclein Overexpression Increases Cytosolic Catecholamine Concentration

  Mosharov, E. V. et al.
  Journal of Neuroscience 26
  (6 September 2006); doi:10.1523/JNEUROSCI.0519-06.2006

  Abstract

• Reduced sodium current in GABAergic interneurons in a mouse model of severe myoclonic epilepsy in infancy

  Frank H Yu, Massimo Mantegazza, Ruth E Westenbroek, Carol A Robbins, Franck Kalume, Kimberly A Burton, William J Spain, G Stanley McKnight, Todd Scheuer and William A Catterall
  Nature Neuroscience 9 1142 - 1149
  (20 August 2006); doi:10.1038/nn1754

  Abstract

• Mice Deficient for the Vesicular Acetylcholine Transporter Are Myasthenic and Have Deficits in Object and Social Recognition

  Prado, V. F. et al.
  Neuron 51
  (7 September 2006); doi:10.1016/j.neuron.2006.08.005

  Abstract

• Regulation of gene expression in the mammalian eye and its relevance to eye disease

  Scheetz, T. E. et al.
  Proceedings of the National Academy of Sciences 103
  (18 September 2006); doi:10.1073/pnas.0602562103

  Abstract

• A Genomic Screen for Modifiers of Tauopathy Identifies Puromycin-Sensitive Aminopeptidase as an Inhibitor of Tau-Induced Neurodegeneration

  Karsten, S. L. et al.
  Neuron 51
  (7 September 2006); doi:10.1016/j.neuron.2006.07.019

  Abstract

• Tumor Necrosis Factor Receptor 1 Is a Negative Regulator of Progenitor Proliferation in Adult Hippocampal Neurogenesis

  Iosif, R. E. et al.
  Journal of Neuroscience 26
  (20 September 2006); doi:10.1523/JNEUROSCI.2723-06.2006

  Abstract

• Neurotrophin-3 Gradients Established by Lentiviral Gene Delivery Promote Short-Distance Axonal Bridging beyond Cellular Grafts in the Injured Spinal Cord

  Taylor, L., Jones, L., Tuszynski, M. H. & Blesch, A.
  Journal of Neuroscience 26
  (20 September 2006); doi:10.1523/JNEUROSCI.0734-06.2006

  Abstract

• Kinase activity of mutant LRRK2 mediates neuronal toxicity

  Wanli W Smith, Zhong Pei, Haibing Jiang, Valina L Dawson, Ted M Dawson and Christopher A Ross
  Nature Neuroscience 9 1231 - 1233
  (17 September 2006); doi:10.1038/nn1776

  Abstract

• Deficiency of hyccin, a newly identified membrane protein, causes hypomyelination and congenital cataract

  Federico Zara, Roberta Biancheri, Claudio Bruno, Laura Bordo, Stefania Assereto, Elisabetta Gazzerro, Federica Sotgia, Xiao Bo Wang, Stefania Gianotti, Silvia Stringara, Marina Pedemonte, Graziella Uziel, Andrea Rossi, Angelo Schenone, Paolo Tortori-Donati, Marjo S van der Knaap, Michael P Lisanti and Carlo Minetti
  Nature Genetics 38 1111 - 1113
  (3 September 2006); doi:10.1038/ng1870

  Abstract