{"doi":"10.1126/science.adp7201","title":"Regulated N-glycosylation controls chaperone function and receptor trafficking","abstract":"One-fifth of human proteins are N-glycosylated in the endoplasmic reticulum (ER) by two oligosaccharyltransferases, OST-A and OST-B. Contrary to the prevailing view of N-glycosylation as a housekeeping function, we identified an ER pathway that modulates the activity of OST-A. Genetic analyses linked OST-A to HSP90B1, an ER chaperone for membrane receptors, and CCDC134, an ER luminal protein. During its translocation into the ER, an N-terminal peptide in HSP90B1 templates the assembly of a translocon complex containing CCDC134 and OST-A that protects HSP90B1 during folding, preventing its hyperglycosylation and degradation. Disruption of this pathway impairs WNT and IGF1R signaling and causes the bone developmental disorder osteogenesis imperfecta. Thus, N-glycosylation can be regulated by specificity factors in the ER to control cell surface receptor signaling and tissue development.","journal":"Science","year":2024,"id":423491,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":24,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.956,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":312313,"name":"Ramin Dubey","orcid":"0000-0002-0687-5831","position":1,"is_corresponding":false},{"id":1034990,"name":"Annie Jen","orcid":"0000-0001-5569-8387","position":2,"is_corresponding":false},{"id":312316,"name":"Ganesh V. Pusapati","orcid":"0000-0002-1406-2566","position":3,"is_corresponding":false},{"id":1218652,"name":"Bharti Singal","orcid":"0000-0002-9482-5251","position":4,"is_corresponding":false},{"id":109552,"name":"Evgenia Shishkova","orcid":"0000-0001-6097-6097","position":5,"is_corresponding":false},{"id":109551,"name":"Katherine A. Overmyer","orcid":"0000-0002-1929-1229","position":6,"is_corresponding":false},{"id":349880,"name":"Valérie Cormier‐Daire","orcid":"0000-0002-2839-9856","position":7,"is_corresponding":false},{"id":700348,"name":"Juliette Fédry","orcid":"0000-0002-6592-4269","position":8,"is_corresponding":false},{"id":286376,"name":"L. Aravind","orcid":"0000-0003-0771-253X","position":9,"is_corresponding":false},{"id":71208,"name":"Joshua J. Coon","orcid":"0000-0002-0004-8253","position":10,"is_corresponding":false},{"id":312321,"name":"Rajat Rohatgi","orcid":"0000-0001-7609-8858","position":11,"is_corresponding":false},{"id":1218651,"name":"Mengxiao Ma","orcid":"0000-0003-4166-6004","position":0,"is_corresponding":true}],"reference_count":61,"raw_metadata":null,"created_at":"2026-07-19T01:57:56.814994Z","pmid":"39509507","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}