Impaired dynamic interaction of axonal endoplasmic reticulum and ribosomes contributes to defective stimulus–response in spinal muscular atrophy

Chunchu Deng, Sebastian Reinhard, Luisa Hennlein, Janna Eilts, Stefan Sachs, Sören Doose, Sibylle Jablonka, Markus Sauer, Mehri Moradi, Michael Sendtner

Translational Neurodegeneration, Vol. 11(1), p. 31 · 2022

DOI: 10.1186/s40035-022-00304-2 ISSN: 2047-9158

Abstract

Axonal degeneration and defects in neuromuscular neurotransmission represent a pathological hallmark in spinal muscular atrophy (SMA) and other forms of motoneuron disease. These pathological changes do not only base on altered axonal and presynaptic architecture, but also on alterations in dynamic movements of organelles and subcellular structures that are not necessarily reflected by static histopathological changes. The dynamic interplay between the axonal endoplasmic reticulum (ER) and ribosomes is essential for stimulus-induced local translation in motor axons and presynaptic terminals. However, it remains enigmatic whether the ER and ribosome crosstalk is impaired in the presynaptic compartment of motoneurons with Smn (survival of motor neuron) deficiency that could contribute to axonopathy and presynaptic dysfunction in SMA.

Keywords

BDNF stimulation Dynamics of ribosomal assembly Presynaptic ER dynamics Spinal muscular atrophy

Cite this article 

@article{deng_impaired_2022,
  title = {Impaired dynamic interaction of axonal endoplasmic reticulum and ribosomes contributes to defective stimulus–response in spinal muscular atrophy},
  volume = {11},
  issn = {2047-9158},
  url = {https://doi.org/10.1186/s40035-022-00304-2},
  doi = {10.1186/s40035-022-00304-2},
  abstract = {Axonal degeneration and defects in neuromuscular neurotransmission represent a pathological hallmark in spinal muscular atrophy (SMA) and other forms of motoneuron disease. These pathological changes do not only base on altered axonal and presynaptic architecture, but also on alterations in dynamic movements of organelles and subcellular structures that are not necessarily reflected by static histopathological changes. The dynamic interplay between the axonal endoplasmic reticulum (ER) and ribosomes is essential for stimulus-induced local translation in motor axons and presynaptic terminals. However, it remains enigmatic whether the ER and ribosome crosstalk is impaired in the presynaptic compartment of motoneurons with Smn (survival of motor neuron) deficiency that could contribute to axonopathy and presynaptic dysfunction in SMA.},
  number = {1},
  urldate = {2022-10-21},
  journal = {Translational Neurodegeneration},
  author = {Deng, Chunchu and Reinhard, Sebastian and Hennlein, Luisa and Eilts, Janna and Sachs, Stefan and Doose, Sören and Jablonka, Sibylle and Sauer, Markus and Moradi, Mehri and Sendtner, Michael},
  year = {2022},
  keywords = {BDNF stimulation, Dynamics of ribosomal assembly, Presynaptic ER dynamics, Spinal muscular atrophy},
  pages = {31},
}
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