http://purl.uniprot.org/citations/31378462 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/31378462 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/31378462 | http://www.w3.org/2000/01/rdf-schema#comment | "Mitochondrial dysfunction and proteostasis failure frequently coexist as hallmarks of neurodegenerative disease. How these pathologies are related is not well understood. Here, we describe a phenomenon termed MISTERMINATE (mitochondrial-stress-induced translational termination impairment and protein carboxyl terminal extension), which mechanistically links mitochondrial dysfunction with proteostasis failure. We show that mitochondrial dysfunction impairs translational termination of nuclear-encoded mitochondrial mRNAs, including complex-I 30kD subunit (C-I30) mRNA, occurring on the mitochondrial surface in Drosophila and mammalian cells. Ribosomes stalled at the normal stop codon continue to add to the C terminus of C-I30 certain amino acids non-coded by mRNA template. C-terminally extended C-I30 is toxic when assembled into C-I and forms aggregates in the cytosol. Enhancing co-translational quality control prevents C-I30 C-terminal extension and rescues mitochondrial and neuromuscular degeneration in a Parkinson's disease model. These findings emphasize the importance of efficient translation termination and reveal unexpected link between mitochondrial health and proteome homeostasis mediated by MISTERMINATE."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.molcel.2019.06.031"xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.molcel.2019.06.031"xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Chen S."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Chen S."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Dong J."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Dong J."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Li Y."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Li Y."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Lim J."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Lim J."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Wu Z."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Wu Z."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Snyder M."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Snyder M."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Lu B."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Lu B."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Bi X."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Bi X."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Brandman O."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Brandman O."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Auburger G."xsd:string |
http://purl.uniprot.org/citations/31378462 | http://purl.uniprot.org/core/author | "Auburger G."xsd:string |