Fas small interfering RNA reduces motoneuron death in amyotrophic lateral sclerosis mice

Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by selective motoneuron death. Understanding of the molecular mechanisms that trigger and regulate motoneuron degeneration could be relevant to ALS and other motoneuron disorders. This study investigates the role of Fas‐linked motoneuron death in the pathogenesis of ALS.

[1]  B. Monia,et al.  Antisense oligonucleotide therapy for neurodegenerative disease. , 2006, The Journal of clinical investigation.

[2]  G. Kollias,et al.  Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia , 2006, Science.

[3]  B. Pettmann,et al.  Chronic activation in presymptomatic amyotrophic lateral sclerosis (ALS) mice of a feedback loop involving Fas, Daxx, and FasL. , 2006, Proceedings of the National Academy of Sciences of the United States of America.

[4]  B. Burkhardt,et al.  Efficient delivery of siRNA into cytokine‐stimulated insulinoma cells silences Fas expression and inhibits Fas‐mediated apoptosis , 2006, FEBS letters.

[5]  M. Shoji,et al.  Prevention of spinal motor neuron death by insulin‐like growth factor‐1 associating with the signal transduction systems in SODG93A transgenic mice , 2005, Journal of neuroscience research.

[6]  I. Kanazawa,et al.  Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA , 2005, Neuroscience Research.

[7]  L. Doughty,et al.  In vivo delivery of caspase-8 or Fas siRNA improves the survival of septic mice. , 2005, Blood.

[8]  T. Heiman-Patterson,et al.  Background and gender effects on survival in the TgN(SOD1-G93A)1Gur mouse model of ALS , 2005, Journal of the Neurological Sciences.

[9]  Inder M Verma,et al.  Targeting BACE1 with siRNAs ameliorates Alzheimer disease neuropathology in a transgenic model , 2005, Nature Neuroscience.

[10]  L. Martin,et al.  Adult Motor Neuron Apoptosis Is Mediated by Nitric Oxide and Fas Death Receptor Linked by DNA Damage and p53 Activation , 2005, The Journal of Neuroscience.

[11]  C. Rooney,et al.  Human cytotoxic T lymphocytes with reduced sensitivity to Fas-induced apoptosis. , 2005, Blood.

[12]  Timothy A. Miller,et al.  Virus‐delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis , 2005, Annals of neurology.

[13]  H. Paulson,et al.  RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[14]  L. Greensmith,et al.  Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model , 2005, Nature Medicine.

[15]  C. Henderson,et al.  Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS , 2005, Nature Medicine.

[16]  F. Natt,et al.  Neurochemical and behavioral consequences of widespread gene knockdown in the adult mouse brain by using nonviral RNA interference. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[17]  C. Donadoni,et al.  Wild-type bone marrow cells ameliorate the phenotype of SOD1-G93A ALS mice and contribute to CNS, heart and skeletal muscle tissues. , 2004, Brain : a journal of neurology.

[18]  J. Lieberman,et al.  Small interfering RNA targeting Fas protects mice against renal ischemia-reperfusion injury. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[19]  A. Rembach,et al.  Antisense peptide nucleic acid targeting GluR3 delays disease onset and progression in the SOD1 G93A mouse model of familial ALS , 2004, Journal of neuroscience research.

[20]  H. Paulson,et al.  RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia , 2004, Nature Medicine.

[21]  L. Bruijn,et al.  Unraveling the mechanisms involved in motor neuron degeneration in ALS. , 2004, Annual review of neuroscience.

[22]  F. Natt,et al.  siRNA relieves chronic neuropathic pain. , 2004, Nucleic acids research.

[23]  J. Glass,et al.  Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man , 2004, Experimental Neurology.

[24]  Chulhee Choi,et al.  Fas ligand/Fas system in the brain: regulator of immune and apoptotic responses , 2004, Brain Research Reviews.

[25]  K. Tokunaga,et al.  Long‐lasting RNAi activity in mammalian neurons , 2004, FEBS letters.

[26]  Minh N. H. Nguyen,et al.  Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice , 2003, Science.

[27]  S. Appel,et al.  Serum anti-Fas antibody levels in amyotrophic lateral sclerosis , 2003, Journal of Neuroimmunology.

[28]  M. Manns,et al.  Caspase 8 small interfering RNA prevents acute liver failure in mice , 2003, Proceedings of the National Academy of Sciences of the United States of America.

[29]  D. Dykxhoorn,et al.  Killing the messenger: short RNAs that silence gene expression , 2003, Nature Reviews Molecular Cell Biology.

[30]  Judy Lieberman,et al.  RNA interference targeting Fas protects mice from fulminant hepatitis , 2003, Nature Medicine.

[31]  Hiroshi Nishimune,et al.  Motoneuron Death Triggered by a Specific Pathway Downstream of Fas Potentiation by ALS-Linked SOD1 Mutations , 2002, Neuron.

[32]  H. Wichterle,et al.  Directed Differentiation of Embryonic Stem Cells into Motor Neurons , 2002, Cell.

[33]  Jeffrey D. Rothstein,et al.  From charcot to lou gehrig: deciphering selective motor neuron death in als , 2001, Nature Reviews Neuroscience.

[34]  A. Fire,et al.  Specific inhibition of gene expression by small double-stranded RNAs in invertebrate and vertebrate systems , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[35]  N. Shibata,et al.  Neuronal nitric oxide synthase immunoreactivity in the spinal cord in amyotrophic lateral sclerosis , 2001, Acta Neuropathologica.

[36]  D. Ferriero,et al.  Delayed Neurodegeneration in Neonatal Rat Thalamus after Hypoxia–Ischemia Is Apoptosis , 2001, The Journal of Neuroscience.

[37]  P. Couratier,et al.  In vitro induction of neuronal apoptosis by anti-Fas antibody-containing sera from amyotrophic lateral sclerosis patients , 2000, Journal of Neuroimmunology.

[38]  P. Stieg,et al.  Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model. , 2000, Science.

[39]  B. Schrank,et al.  Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III. , 2000, Human molecular genetics.

[40]  P. Ince,et al.  Expression of nitric oxide synthase isoforms in spinal cord in amyotrophic lateral sclerosis , 2000 .

[41]  B. Pettmann,et al.  Programmed Cell Death of Embryonic Motoneurons Triggered through the FAS Death Receptor , 1999, The Journal of cell biology.

[42]  J. Wands,et al.  P53- and CD95-associated apoptosis in neurodegenerative diseases. , 1998, Laboratory investigation; a journal of technical methods and pathology.

[43]  M. Gurney,et al.  Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. , 1994, Science.

[44]  D. C. Carter,et al.  Atomic structure and chemistry of human serum albumin , 1993, Nature.

[45]  J. Haines,et al.  Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis , 1993, Nature.

[46]  W. Bradley,et al.  Mitochondrial involvement in amyotrophic lateral sclerosis , 2007, Molecular Neurobiology.

[47]  B. Pettmann,et al.  Cardiotrophin‐1 requires LIFRβ to promote survival of mouse motoneurons purified by a novel technique , 1999, Journal of neuroscience research.

[48]  G. Wilkin,et al.  Neural cell culture: a practical approach , 1995 .