Cells are like factories, only smaller: Tiny machines assemble large proteins from very small molecules. But where do the machines come from? Researchers at the University of Würzburg have now found an answer to this question.
If the assembly fails to produce a fully functional RNA-protein complex, diseases may result. This is what happens in the case of spinal muscular atrophy (SMA). In SMA patients, the helpers are unable to produce the required number of assembly machines, which results in a lack of important RNA-protein complexes. SMA is characterised by the loss of motor neurones in the spinal cord and brainstem, resulting in severe paralysis and death.
Ashwin Chari, Monika M. Golas, Michael Klingenhäger, Nils Neuenkirchen, Bjoern Sander, Clemens Englbrecht, Albert Sickmann, Holger Stark, and Utz Fischer (2008), An Assembly Chaperone Collaborates with the SMN Complex to Generate Spliceosomal SnRNP. Cell, DOI 10.1016/j.cell.2008.09.020