Amyotrophe Lateralsklerose

[1]  O. Hardiman,et al.  A revision of the El Escorial criteria - 2015 , 2015, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[2]  Brittany N. Lasseigne,et al.  Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways , 2015, Science.

[3]  T. Wieland,et al.  Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia , 2015, Nature Neuroscience.

[4]  Christian Burkhardt,et al.  The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: A cross-sectional comparison of established screening tools in a German-Swiss population , 2015, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[5]  G. Cruccu,et al.  Small-fibre neuropathy related to bulbar and spinal-onset in patients with ALS , 2015, Journal of Neurology.

[6]  C. Shaw,et al.  TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets , 2015, Neurotherapeutics.

[7]  C. Shaw,et al.  TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets , 2015, Neurotherapeutics.

[8]  H. Benali,et al.  Electrophysiological and spinal imaging evidences for sensory dysfunction in amyotrophic lateral sclerosis , 2015, BMJ Open.

[9]  S. Petri,et al.  Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study , 2015, Journal of Neurology.

[10]  J. Ravits,et al.  Focality, stochasticity and neuroanatomic propagation in ALS pathogenesis , 2014, Experimental Neurology.

[11]  H. Braak,et al.  Diffusion tensor imaging analysis of sequential spreading of disease in amyotrophic lateral sclerosis confirms patterns of TDP-43 pathology. , 2014, Brain : a journal of neurology.

[12]  Jochen H Weishaupt,et al.  Genetik und Neurochemische Biomarker bei Amyotropher Lateralsklerose und Frontotemporaler Lobärdegeneration , 2014, Aktuelle Neurologie.

[13]  G. Nagel,et al.  Incidence and Geographical Variation of Amyotrophic Lateral Sclerosis (ALS) in Southern Germany – Completeness of the ALS Registry Swabia , 2014, PloS one.

[14]  M. Grossman,et al.  Deficits in sentence expression in amyotrophic lateral sclerosis , 2015, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[15]  M. Ogino,et al.  Syntactic Comprehension in Patients with Amyotrophic Lateral Sclerosis , 2014, Behavioural neurology.

[16]  L. Petrucelli,et al.  Casein Kinase II Induced Polymerization of Soluble TDP-43 into Filaments Is Inhibited by Heat Shock Proteins , 2014, PloS one.

[17]  U. Sengupta,et al.  TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo. , 2014, Human molecular genetics.

[18]  J. Trojanowski,et al.  Therapeutic modulation of eIF2α-phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models , 2013, Nature Genetics.

[19]  John Q. Trojanowski,et al.  Amyotrophic lateral sclerosis—a model of corticofugal axonal spread , 2013, Nature Reviews Neurology.

[20]  A. Ludolph,et al.  High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[21]  G. Comi,et al.  Autosomal Dominant Frontotemporal Lobar Degeneration Due to the C9ORF72 Hexanucleotide Repeat Expansion: Late-Onset Psychotic Clinical Presentation , 2013, Biological Psychiatry.

[22]  D. Cleveland,et al.  Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis , 2013, Neuron.

[23]  Murray Grossman,et al.  Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis , 2013, Annals of neurology.

[24]  L. Goldstein,et al.  Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment , 2013, The Lancet Neurology.

[25]  T. Anderson,et al.  Eye movements in patients with neurodegenerative disorders , 2013, Nature Reviews Neurology.

[26]  J. Kassubek,et al.  Parkinson’s disease-like midbrain hyperechogenicity is frequent in amyotrophic lateral sclerosis , 2013, Journal of Neurology.

[27]  P. Andersen,et al.  Progressive aphasia as the presenting symptom in a patient with amyotrophic lateral sclerosis with a novel mutation in the OPTN gene , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[28]  C. Broeckhoven,et al.  The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum. , 2012 .

[29]  K. Tsai,et al.  Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43 , 2012, Proceedings of the National Academy of Sciences.

[30]  A. Chiò,et al.  Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV , 2012, Neurology.

[31]  David T. Jones,et al.  Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72 , 2012, Brain : a journal of neurology.

[32]  David Heckerman,et al.  A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD , 2011, Neuron.

[33]  Bruce L. Miller,et al.  Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS , 2011, Neuron.

[34]  I. Katona,et al.  Small-fiber neuropathy in patients with ALS , 2011, Neurology.

[35]  J. Kassubek,et al.  Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis , 2011, Journal of Neurology.

[36]  Hiroaki Suzuki,et al.  TDP-43-induced Death Is Associated with Altered Regulation of BIM and Bcl-xL and Attenuated by Caspase-mediated TDP-43 Cleavage* , 2011, The Journal of Biological Chemistry.

[37]  S. Akbarian,et al.  The C-Terminal TDP-43 Fragments Have a High Aggregation Propensity and Harm Neurons by a Dominant-Negative Mechanism , 2010, PloS one.

[38]  Susan A. King,et al.  The Disturbance of Gaze in Progressive Supranuclear Palsy: Implications for Pathogenesis , 2010, Front. Neur..

[39]  H. Ichikawa.  [Language disorders in ALS/FTLD]. , 2010, Rinshō shinkeigaku Clinical neurology.

[40]  Takeo Kato,et al.  Mutations of optineurin in amyotrophic lateral sclerosis , 2010, Nature.

[41]  D. Piwnica-Worms,et al.  Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease , 2009, The Journal of cell biology.

[42]  V. Meininger,et al.  Extrapyramidal stiffness in patients with amyotrophic lateral sclerosis , 2009, Movement disorders : official journal of the Movement Disorder Society.

[43]  J. Haines,et al.  Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis , 2009, Science.

[44]  Xun Hu,et al.  Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6 , 2009, Science.

[45]  B. McConkey,et al.  TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis , 2008, Nature Genetics.

[46]  J. Morris,et al.  TDP‐43 A315T mutation in familial motor neuron disease , 2008, Annals of neurology.

[47]  V. Meininger,et al.  Dyslipidemia is a protective factor in amyotrophic lateral sclerosis , 2008, Neurology.

[48]  Xun Hu,et al.  TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis , 2008, Science.

[49]  T. Williams.  Effect of non-invasive ventilation on survival, quality of life, respiratory function and cognition: a review of the literature. , 2007, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[50]  Bruce L. Miller,et al.  Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis , 2006, Science.

[51]  J. Kassubek,et al.  Emotional responding in amyotrophic lateral sclerosis , 2005, Journal of Neurology.

[52]  A. Ludolph,et al.  Cognitive function in bulbar– and spinal–onset amyotrophic lateral sclerosis , 2005, Journal of Neurology.

[53]  P. Howley,et al.  The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome. , 2000, Molecular cell.

[54]  D. Sautereau,et al.  Nutritional status is a prognostic factor for survival in ALS patients , 1999, Neurology.

[55]  S. Tsuji,et al.  Neuropathology of sporadic amyotrophic lateral sclerosis of long duration , 1997, Journal of the Neurological Sciences.

[56]  C Kennard,et al.  Oculomotor function in amyotrophic lateral sclerosis: Evidence for frontal impairment , 1995, Annals of neurology.

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

[58]  J. Antel,et al.  Eye movements in amyotrophic lateral sclerosis. , 1982, Archives of neurology.

[59]  W. Engel,et al.  Amyotrophic lateral sclerosis: metabolism of central monoamines and treatment with L-dopa. , 1971, Transactions of the American Neurological Association.