{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Ma W"],"funding":["Howard Hughes Medical Institute","Memorial Sloan-Kettering Cancer Center"],"pagination":["e26624"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC5464768"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["6"],"pubmed_abstract":["Native cargo proteins exit the endoplasmic reticulum (ER) in COPII-coated vesicles, whereas resident and misfolded proteins are substantially excluded from vesicles by a retention mechanism that remains unresolved. We probed the ER retention process using the proteostasis regulator 4-phenylbutyrate (4-PBA), which we show targets COPII protein to reduce the stringency of retention. 4-PBA competes with p24 proteins to bind COPII. When p24 protein uptake is blocked, COPII vesicles package resident proteins and an ER-trapped mutant LDL receptor. We further show that 4-PBA triggers the secretion of a KDEL-tagged luminal resident, implying that a compromised retention mechanism causes saturation of the KDEL retrieval system. The results indicate that stringent ER retention requires the COPII coa"],"journal":["eLife"],"pubmed_title":["ER retention is imposed by COPII protein sorting and attenuated by 4-phenylbutyrate."],"pmcid":["PMC5464768"],"funding_grant_id":["P30-CA008748"],"pubmed_authors":["Goldberg E","Goldberg J","Ma W"],"additional_accession":[]},"is_claimable":false,"name":"ER retention is imposed by COPII protein sorting and attenuated by 4-phenylbutyrate.","description":"Native cargo proteins exit the endoplasmic reticulum (ER) in COPII-coated vesicles, whereas resident and misfolded proteins are substantially excluded from vesicles by a retention mechanism that remains unresolved. We probed the ER retention process using the proteostasis regulator 4-phenylbutyrate (4-PBA), which we show targets COPII protein to reduce the stringency of retention. 4-PBA competes with p24 proteins to bind COPII. When p24 protein uptake is blocked, COPII vesicles package resident proteins and an ER-trapped mutant LDL receptor. We further show that 4-PBA triggers the secretion of a KDEL-tagged luminal resident, implying that a compromised retention mechanism causes saturation of the KDEL retrieval system. The results indicate that stringent ER retention requires the COPII coa","dates":{"release":"2017-01-01T00:00:00Z","publication":"2017 Jun","modification":"2025-05-31T23:29:52.317Z","creation":"2025-05-31T23:29:52.317Z"},"accession":"S-EPMC5464768","cross_references":{"pubmed":["28594326"],"doi":["10.7554/eLife.26624"]}}