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Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCF(Slmb) degron
Spinal muscular atrophy (SMA) is caused by homozygous mutations in human SMN1. Expression of a duplicate gene (SMN2) primarily results in skipping of exon 7 and production of an unstable protein isoform, SMNΔ7. Although SMN2 exon skipping is the principal contributor to SMA severity, mechanisms gove...
Autores principales: | , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society for Cell Biology
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5909936/ https://www.ncbi.nlm.nih.gov/pubmed/29167380 http://dx.doi.org/10.1091/mbc.E17-11-0627 |