Regulation of the ER stress response by a mitochondrial microprotein. Chu, Q., Martinez, T. F, Novak, S. W., Donaldson, C. J, Tan, D., Vaughan, J. M, Chang, T., Diedrich, J. K, Andrade, L., Kim, A., Zhang, T., Manor, U., & Saghatelian, A. Nature Communications, 10(1):4883, 2019. ![Regulation of the ER stress response by a mitochondrial microprotein [link]](https://bibbase.org/img/filetypes/link.svg "link")
Paper doi abstract bibtex Cellular homeostasis relies on having dedicated and coordinated responses to a variety of stresses. The accumulation of unfolded proteins in the endoplasmic reticulum (ER) is a common stress that triggers a conserved pathway called the unfolded protein response (UPR) that mitigates damage, and dysregulation of UPR underlies several debilitating diseases. Here, we discover that a previously uncharacterized 54-amino acid microprotein PIGBOS regulates UPR. PIGBOS localizes to the mitochondrial outer membrane where it interacts with the ER protein CLCC1 at ER–mitochondria contact sites. Functional studies reveal that the loss of PIGBOS leads to heightened UPR and increased cell death. The characterization of PIGBOS reveals an undiscovered role for a mitochondrial protein, in this case a microprotein, in the regulation of UPR originating in the ER. This study demonstrates microproteins to be an unappreciated class of genes that are critical for inter-organelle communication, homeostasis, and cell survival.
@article{Chu2019,
abstract = {Cellular homeostasis relies on having dedicated and coordinated responses to a variety of stresses. The accumulation of unfolded proteins in the endoplasmic reticulum (ER) is a common stress that triggers a conserved pathway called the unfolded protein response (UPR) that mitigates damage, and dysregulation of UPR underlies several debilitating diseases. Here, we discover that a previously uncharacterized 54-amino acid microprotein PIGBOS regulates UPR. PIGBOS localizes to the mitochondrial outer membrane where it interacts with the ER protein CLCC1 at ER–mitochondria contact sites. Functional studies reveal that the loss of PIGBOS leads to heightened UPR and increased cell death. The characterization of PIGBOS reveals an undiscovered role for a mitochondrial protein, in this case a microprotein, in the regulation of UPR originating in the ER. This study demonstrates microproteins to be an unappreciated class of genes that are critical for inter-organelle communication, homeostasis, and cell survival.},
author = {Chu, Qian and Martinez, Thomas F and Novak, Sammy Weiser and Donaldson, Cynthia J and Tan, Dan and Vaughan, Joan M and Chang, Tina and Diedrich, Jolene K and Andrade, Leo and Kim, Andrew and Zhang, Tong and Manor, Uri and Saghatelian, Alan},
doi = {10.1038/s41467-019-12816-z},
issn = {2041-1723},
journal = {Nature Communications},
number = {1},
pages = {4883},
title = {{Regulation of the ER stress response by a mitochondrial microprotein}},
url = {https://doi.org/10.1038/s41467-019-12816-z},
volume = {10},
year = {2019}
}
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PIGBOS localizes to the mitochondrial outer membrane where it interacts with the ER protein CLCC1 at ER–mitochondria contact sites. Functional studies reveal that the loss of PIGBOS leads to heightened UPR and increased cell death. The characterization of PIGBOS reveals an undiscovered role for a mitochondrial protein, in this case a microprotein, in the regulation of UPR originating in the ER. 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