Motor neuron disease articles within Nature

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• Article
  14 February 2024 | Open Access

  A model of human neural networks reveals NPTX2 pathology in ALS and FTLD

  A neural stem cell culture system derived from induced pluripotent stem cells forms a network of synaptically connected and electrophysiologically active neurons that were used as a model system to identify a mechanism of TDP-43-induced neurodegeneration.

  • Marian Hruska-Plochan
  • , Vera I. Wiersma
  •  & Magdalini Polymenidou

• Article | 22 July 2019

  Potential roles of gut microbiome and metabolites in modulating ALS in mice

  A study of the functional microbiome in a mouse model of ALS shows that several gut bacteria may modulate the severity of the disease.

  • Eran Blacher
  • , Stavros Bashiardes
  •  & Eran Elinav

• Article | 31 October 2018

  TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle

  Cytoplasmic, amyloid-like oligomeric assemblies that contain TDP-43 are increased in damaged tissues with elevated regeneration, thereby enhancing the possibility of amyloid fibre formation and/or aggregation of TDP-43 in disease.

  • Thomas O. Vogler
  • , Joshua R. Wheeler
  •  & Roy Parker

• Letter | 12 April 2017

  Antisense oligonucleotide therapy for spinocerebellar ataxia type 2

  Antisense oligonucleotides against ATXN2 improved motor neuron function and restored firing frequency in cerebellar Purkinje cells in mouse models of spinocerebellar ataxia type 2.

  • Daniel R. Scoles
  • , Pratap Meera
  •  & Stefan M. Pulst

• Letter | 26 August 2015

  GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

  An unbiased genetic screen in Drosophila expressing G₄C₂-repeat-containing transcripts (repeats that in human cause pathogenesis in C9orf72-related neurological disease) finds genes that encode components of the nuclear pore and nucleocytoplasmic transport machinery, and reveals that G₄C₂ expanded-repeat-induced alterations in nucleocytoplasmic transport contribute to C9orf72 pathology and neurodegeneration.

  • Brian D. Freibaum
  • , Yubing Lu
  •  & J. Paul Taylor

• Letter | 05 October 2011

  Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model

  • Yimin Hua
  • , Kentaro Sahashi
  •  & Adrian R. Krainer