Ontology highlight
ABSTRACT:
SUBMITTER: Wan L
PROVIDER: S-EPMC2867055 | biostudies-literature | 2008 Jul
REPOSITORIES: biostudies-literature
Wan Lili L Ottinger Elizabeth E Cho Sungchan S Dreyfuss Gideon G
Molecular cell 20080701 2
The SMN complex is essential for the biogenesis of small nuclear ribonucleoproteins (snRNPs), the major constituents of the spliceosome. Deficiency in functional SMN protein causes spinal muscular atrophy, a common motor neuron degenerative disease of severity commensurate with SMN levels and, correspondingly, snRNP assembly decreases. We developed a high-throughput screen for snRNP assembly modifiers and discovered that reactive oxygen species (ROS) inhibit SMN-complex activity in a dose-depend ...[more]