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...

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Detalles Bibliográficos
Autores principales: Gray, Kelsey M., Kaifer, Kevin A., Baillat, David, Wen, Ying, Bonacci, Thomas R., Ebert, Allison D., Raimer, Amanda C., Spring, Ashlyn M., Have, Sara ten, Glascock, Jacqueline J., Gupta, Kushol, Van Duyne, Gregory D., Emanuele, Michael J., Lamond, Angus I., Wagner, Eric J., Lorson, Christian L., Matera, A. Gregory
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The American Society for Cell Biology 2018
Materias:
Articles
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

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