Article
Pathogenic Signal Sequence Mutations in Progranulin Disrupt SRP Interactions Required for mRNA Stability.
Cell reports - 5 Jun 2018
Pinarbasi Emile S, Karamyshev Andrey L, Tikhonova Elena B, Wu I-Hui, Hudson Henry, Thomas Philip J
Abstract excerpt
Cells have evolved quality control pathways to prevent the accumulation of improperly localized proteins, which are often toxic. One of these pathways, regulation of aberrant protein production (RAPP), recognizes aberrant secretory proteins during translation and degrades the associated mRNA. Here, we demonstrate endogenous RAPP substrates. Haploinsufficiency of the secretory protein progranulin (GRN) is...
Topics
- Argonaute Proteins
- HeLa Cells
- Humans
- Mutant Proteins
- Mutation
- Progranulins
- Protein Biosynthesis
- Protein Sorting Signals
- RNA Stability
- RNA, Messenger
- Signal Recognition Particle