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PMID:15280428
| Citation |
Yoneda, T, Benedetti, C, Urano, F, Clark, SG, Harding, HP and Ron, D (2004) Compartment-specific perturbation of protein handling activates genes encoding mitochondrial chaperones. J. Cell. Sci. 117:4055-66 |
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| Abstract |
Protein folding in the mitochondria is assisted by nuclear-encoded compartment-specific chaperones but regulation of the expression of their encoding genes is poorly understood. We found that the mitochondrial matrix HSP70 and HSP60 chaperones, encoded by the Caenorhabditis elegans hsp-6 and hsp-60 genes, were selectively activated by perturbations that impair assembly of multi-subunit mitochondrial complexes or by RNAi of genes encoding mitochondrial chaperones or proteases, which lead to defective protein folding and processing in the organelle. hsp-6 and hsp-60 induction was specific to perturbed mitochondrial protein handling, as neither heat-shock nor endoplasmic reticulum stress nor manipulations that impair mitochondrial steps in intermediary metabolism or ATP synthesis activated the mitochondrial chaperone genes. These observations support the existence of a mitochondrial unfolded protein response that couples mitochondrial chaperone gene expression to changes in the protein handling environment in the organelle. |
| Links |
PubMed Online version:10.1242/jcs.01275 |
| Keywords |
Animals; Caenorhabditis elegans; Cell Compartmentation/physiology; Chaperonin 60/chemistry; Chaperonin 60/genetics; Chaperonin 60/metabolism; Endoplasmic Reticulum/metabolism; Energy Metabolism/physiology; Gene Expression Regulation/physiology; HSP70 Heat-Shock Proteins/chemistry; HSP70 Heat-Shock Proteins/genetics; HSP70 Heat-Shock Proteins/metabolism; Heat-Shock Response/physiology; Intracellular Membranes/metabolism; Macromolecular Substances/metabolism; Mitochondria/genetics; Mitochondria/metabolism; Molecular Chaperones/chemistry; Molecular Chaperones/genetics; Molecular Chaperones/metabolism; Oxidative Stress/physiology; Protein Folding; RNA Interference; Transcriptional Activation/physiology |
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Significance
Annotations
| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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