Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs.
Spinal muscular atrophy (SMA) is a motor neuron disease caused by reduced levels of the survival motor neuron (SMN) protein. SMN together with Gemins2-8 and unrip proteins form a macromolecular complex that functions in the assembly of small nuclear ribonucleoproteins (snRNPs) of both the major and...
Main Authors: | , , , , , |
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Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2007-09-01
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Series: | PLoS ONE |
Online Access: | http://europepmc.org/articles/PMC1976558?pdf=render |