All Stories

  1. Cholesterol dyshomeostasis in amyotrophic lateral sclerosis: cause, consequence, or epiphenomenon?
  2. TDP-43 mediates SREBF2-regulated gene expression required for oligodendrocyte myelination
  3. TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells
  4. Loss of TDP-43 in astrocytes leads to motor deficits by triggering A1-like reactive phenotype and triglial dysfunction
  5. Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult hippocampal neurogenesis
  6. The vulnerability of motor and frontal cortex-dependent behaviors in mice expressing ALS-linked mutation in TDP-43
  7. FUS-mediated dysregulation of Sema5a, an autism-related gene, in FUS mice with hippocampus-dependent cognitive deficits
  8. C9orf72 intermediate repeats are associated with corticobasal degeneration, increased C9orf72 expression and disruption of autophagy
  9. Elevated FUS levels by overriding its autoregulation produce gain-of-toxicity properties that disrupt protein and RNA homeostasis
  10. Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis
  11. The ALS-FTD-linked gene product, C9orf72, regulates neuronal morphogenesis via autophagy
  12. ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS
  13. Cell-autonomous requirement of TDP-43, an ALS/FTD signature protein, for oligodendrocyte survival and myelination
  14. Ooplasmic flow cooperates with transport and anchorage in Drosophila oocyte posterior determination
  15. Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients
  16. Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions
  17. Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis
  18. Evaluation of NADPH oxidases as drug targets in a mouse model of familial amyotrophic lateral sclerosis
  19. FUS-linked essential tremor associated with motor dysfunction in Drosophila
  20. Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs
  21. Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)
  22. Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS
  23. Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS
  24. ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP
  25. Muscle Expression of Mutant Androgen Receptor Accounts for Systemic and Motor Neuron Disease Phenotypes in Spinal and Bulbar Muscular Atrophy
  26. Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration
  27. Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
  28. Fused in Sarcoma (FUS) Protein Lacking Nuclear Localization Signal (NLS) and Major RNA Binding Motifs Triggers Proteinopathy and Severe Motor Phenotype in Transgenic Mice
  29. ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43
  30. Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
  31. Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
  32. ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS
  33. Microtubule binding by dynactin is required for microtubule organization but not cargo transport
  34. Proteomic Characterization of Messenger Ribonucleoprotein Complexes Bound to Nontranslated or Translated Poly(A) mRNAs in the Rat Cerebral Cortex
  35. Transport of Drosophila fragile X mental retardation protein-containing ribonucleoprotein granules by kinesin-1 and cytoplasmic dynein
  36. A Receptor for Activated C Kinase Is Part of Messenger Ribonucleoprotein Complexes Associated with PolyA-mRNAs in Neurons
  37. Characterization of Granular Particles Isolated from Postsynaptic Densities