PXD014890

PXD014890 is an original dataset announced via ProteomeXchange.

Title	Regulation of the ER stress response by a mitochondrial microprotein
Description	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 unprecedented 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.
HostingRepository	PRIDE
AnnounceDate	2019-11-12
AnnouncementXML	Submission_2019-11-11_18:32:52.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Jolene Diedrich
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2019-08-02 02:42:44	ID requested
⏵ 1	2019-11-11 18:32:53	announced

Chu Q, Martinez TF, Novak SW, Donaldson CJ, Tan D, Vaughan JM, Chang T, Diedrich JK, Andrade L, Kim A, Zhang T, Manor U, Saghatelian A, Regulation of the ER stress response by a mitochondrial microprotein. Nat Commun, 10(1):4883(2019) [pubmed]

submitter keyword: ER stress response, mitochondrial, microprotein, smORFs

Alan Saghatelian
contact affiliation	PBLA, Salk Institute, La Jolla, CA, USA
contact email	asaghatelian@salk.edu
lab head
Jolene Diedrich
contact affiliation	Salk
contact email	jdiedrich@salk.edu
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2019/11/PXD014890

PRIDE project URI

• PRIDE
  1. PXD014890
     1. Label: PRIDE project
     2. Name: Regulation of the ER stress response by a mitochondrial microprotein