Is good housekeeping the key to motor neuron survival?

Kevin Talbot; Kay E Davies

doi:10.1016/j.cell.2008.05.002

Is good housekeeping the key to motor neuron survival?

Cell. 2008 May 16;133(4):572-4. doi: 10.1016/j.cell.2008.05.002.

Authors

Kevin Talbot¹, Kay E Davies

Affiliation

¹ MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK. kevin.talbot@clneuro.ox.ac.uk

PMID: 18485864
DOI: 10.1016/j.cell.2008.05.002

Abstract

Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. However, it is unclear why loss of SMN and altered snRNP assembly only seem to affect motor neurons. Reporting in this issue, Zhang et al. (2008) challenge prior assumptions about the housekeeping function of SMN and demonstrate that loss of SMN leads to highly tissue-specific effects on splicing.

Publication types

Comment

MeSH terms

Animals
Cyclic AMP Response Element-Binding Protein / metabolism*
Humans
Mice
Motor Neurons / metabolism*
Muscular Atrophy, Spinal / metabolism
Nerve Tissue Proteins / metabolism*
RNA Splicing*
RNA-Binding Proteins / metabolism*
Ribonucleoproteins, Small Nuclear / metabolism
SMN Complex Proteins

Substances

Cyclic AMP Response Element-Binding Protein
Nerve Tissue Proteins
RNA-Binding Proteins
Ribonucleoproteins, Small Nuclear
SMN Complex Proteins