The role of RNA processing in the pathogenesis of motor neuron degeneration

Dirk Bäumer; Olaf Ansorge; Mara Almeida; Kevin Talbot

doi:10.1017/S1462399410001523

The role of RNA processing in the pathogenesis of motor neuron degeneration

Published online by Cambridge University Press: 20 July 2010

Mara Almeida and

Dirk Bäumer: Affiliation:
MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3QX, UK. Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital, Oxford, OX3 9DU, UK.
Olaf Ansorge: Affiliation:
Department of Neuropathology, John Radcliffe Hospital, Oxford, OX3 9DU, UK.
Mara Almeida: Affiliation:
MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3QX, UK.
Kevin Talbot*: Affiliation:
MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, OX1 3QX, UK. Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital, Oxford, OX3 9DU, UK.
*: *Corresponding author: Kevin Talbot, MRC Functional Genomics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, South Parks Road, Oxford, OX1 3QX, UK. E-mail: kevin.talbot@clneuro.ox.ac.uk

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Abstract

Motor neurons are large, highly polarised cells with very long axons and a requirement for precise spatial and temporal gene expression. Neurodegenerative disorders characterised by selective motor neuron vulnerability include various forms of amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). A rapid expansion in knowledge on the pathophysiology of motor neuron degeneration has occurred in recent years, largely through the identification of genes leading to familial forms of ALS and SMA. The major emerging theme is that motor neuron degeneration can result from mutation in genes that encode factors important for ribonucleoprotein biogenesis and RNA processing, including splicing regulation, transcript stabilisation, translational repression and localisation of mRNA. Complete understanding of how these pathways interact and elucidation of specialised mechanisms for mRNA targeting and processing in motor neurons are likely to produce new targets for therapy in ALS and related disorders.

Type: Review Article
Information: Expert Reviews in Molecular Medicine , Volume 12 , January 2010 , e21

DOI: https://doi.org/10.1017/S1462399410001523 [Opens in a new window]
Copyright: Copyright © Cambridge University Press 2010

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The role of RNA processing in the pathogenesis of motor neuron degeneration

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The role of RNA processing in the pathogenesis of motor neuron degeneration

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