PMID:16816413

Citation	Benedetti, C, Haynes, CM, Yang, Y, Harding, HP and Ron, D (2006) Ubiquitin-like protein 5 positively regulates chaperone gene expression in the mitochondrial unfolded protein response. Genetics 174:229-39
Abstract	Perturbation of the protein-folding environment in the mitochondrial matrix selectively upregulates the expression of nuclear genes encoding mitochondrial chaperones. To identify components of the signal transduction pathway(s) mediating this mitochondrial unfolded protein response (UPR(mt)), we first isolated a temperature-sensitive mutation (zc32) that conditionally activates the UPR(mt) in C. elegans and subsequently searched for suppressors by systematic inactivation of genes. RNAi of ubl-5, a gene encoding a ubiquitin-like protein, suppresses activation of the UPR(mt) markers hsp-60::gfp and hsp-6::gfp by the zc32 mutation and by other manipulations that promote mitochondrial protein misfolding. ubl-5 (RNAi) inhibits the induction of endogenous mitochondrial chaperone encoding genes hsp-60 and hsp-6 and compromises the ability of animals to cope with mitochondrial stress. Mitochondrial morphology and assembly of multi-subunit mitochondrial complexes of biotinylated proteins are also perturbed in ubl-5(RNAi) worms, indicating that UBL-5 also counteracts physiological levels of mitochondrial stress. Induction of mitochondrial stress promotes accumulation of GFP-tagged UBL-5 in nuclei of transgenic worms, suggesting that UBL-5 effects a nuclear step required for mounting a response to the threat of mitochondrial protein misfolding.
Links	PubMed PMC1569816 Online version:10.1534/genetics.106.061580
Keywords	Animals; Animals, Genetically Modified; Caenorhabditis elegans/metabolism; Caenorhabditis elegans Proteins/metabolism; Cell Nucleus/drug effects; Cell Nucleus/metabolism; Gene Expression Regulation/drug effects; Mitochondria/drug effects; Mitochondria/metabolism; Mitochondrial Proteins/metabolism; Models, Biological; Molecular Chaperones/metabolism; Mutation; Protein Folding; RNA Interference; RNA, Small Interfering/adverse effects; Tissue Distribution/drug effects; Ubiquitins/metabolism; Ubiquitins/physiology
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