New insights into how motor neurones live and die in motor neurone disease

A new study from the Gillingwater lab, working in collaboration with colleagues from the University of Trento in Italy, has shown that a key protein involved in the childhood form of motor neuron disease - known as Spinal Muscular Atrophy (SMA) – plays an important role in regulating the generation of new proteins in neurons (via a process known as translation).

Cell Reports Journal Cover

The study revealed that the SMN protein regulates protein translation by influencing one major element of the machinery responsible for converting RNA into protein in cells, known as ribosomes. Experiments in a mouse model of SMA demonstrated that defects in protein translation and ribosomes are an important, but reversible, factor that can contribute to the breakdown and loss of motor neurons in motor neuron disease.

This article was published in Cell Reports and is free to download.

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Journal article: In Vivo Translatome Profiling in Spinal Muscular Atrophy Reveals a Role for SMN Protein in Ribosome Biology