Protein interaction analysis of senataxin and the ALS4 L389S mutant yields insights into senataxin post-translational modification and uncovers mutant-specific binding with a brain cytoplasmic RNA-encoded peptide.
Senataxin is a large 303 kDa protein linked to neuron survival, as recessive mutations cause Ataxia with Oculomotor Apraxia type 2 (AOA2), and dominant mutations cause amyotrophic lateral sclerosis type 4 (ALS4). Senataxin contains an amino-terminal protein-interaction domain and a carboxy-terminal...
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Main Authors: | Craig L Bennett, Yingzhang Chen, Marissa Vignali, Russell S Lo, Amanda G Mason, Asli Unal, Nabiha P Huq Saifee, Stanley Fields, Albert R La Spada |
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Format: | article |
Language: | EN |
Published: |
Public Library of Science (PLoS)
2013
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Online Access: | https://doaj.org/article/93a9bf01b1ad458a8e692455b86ad31c |
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