The mitochondrial UPR - Protecting organelle protein homeostasis Journal Article
| Authors: | Haynes, C. M.; Ron, D. |
| Article Title: | The mitochondrial UPR - Protecting organelle protein homeostasis |
| Abstract: | Mitochondria are required for numerous essential metabolic processes including the regulation of apoptosis; therefore, proper maintenance of the mitochondrial proteome is crucial. The protein-folding environment in mitochondria is challenged by organelle architecture, the presence of reactive oxygen species and the difficulties associated with assembly of the electron transport chain, which consists of components encoded by both the mitochondrial and the nuclear genomes. Mitochondria have dedicated molecular chaperones and proteases that promote proper protein folding, complex assembly and quality control. Work in cultured mammalian cells and Caenorhabditis elegans has yielded clues to the mechanisms linking perturbations in the protein-folding environment in the mitochondrial matrix to the expression of nuclear genes encoding mitochondrial proteins. Here, we review the current knowledge of this mitochondrial unfolded protein response (UPRmt), compare it with the better understood UPR of the endoplasmic reticulum and highlight its potential impact on development and disease. |
| Keywords: | signal transduction; unclassified drug; dna binding protein; nonhuman; note; neoplasm; mammalia; transcription initiation; cell protein; protein; endoplasmic reticulum; abc transporter; caenorhabditis elegans; protein folding; homeostasis; cellular stress response; mitochondria; mitochondrion; endopeptidase clp; heat shock; mammal cell; molecular chaperones; proteases; protein homeostasis; endopeptidase clpx; growth arrest and dna damage inducible protein 153; heat shock protein 60; leucine zipper protein; protein dve 1; protein haf 1; protein ubl 5; protein zc376 7; friedreich ataxia; spastic paraplegia; unfolded protein response |
| Journal Title: | Journal of Cell Science |
| Volume: | 123 |
| Issue: | Pt. 22 |
| ISSN: | 0021-9533 |
| Publisher: | Company of Biologists |
| Date Published: | 2010-11-15 |
| Start Page: | 3849 |
| End Page: | 3855 |
| Language: | English |
| DOI: | 10.1242/jcs.075119 |
| PROVIDER: | scopus |
| PUBMED: | 21048161 |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 1" - "Export Date: 20 April 2011" - "CODEN: JNCSA" - "Source: Scopus" |
