{"doi":"10.1007/s10238-025-01978-4","title":"Dysregulation of the ubiquitin-proteasome system in von Hippel-Lindau syndrome: molecular insights and clinical perspectives","abstract":null,"journal":"Clinical and Experimental Medicine","year":2025,"id":615653,"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":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":816285,"name":"Rui Wang","orcid":"0000-0001-8007-2503","position":1,"is_corresponding":false},{"id":522343,"name":"Lu Li","orcid":"0009-0000-3747-6203","position":2,"is_corresponding":false},{"id":543847,"name":"Lili Wang","orcid":"0000-0003-3321-215X","position":3,"is_corresponding":false},{"id":1142987,"name":"Xingwang Chen","orcid":"0000-0003-2681-8436","position":4,"is_corresponding":false},{"id":1586973,"name":"Xiaomei Nie","orcid":null,"position":5,"is_corresponding":false},{"id":1066548,"name":"Jia Meng","orcid":"0000-0002-8121-8882","position":6,"is_corresponding":false},{"id":1586974,"name":"Shanjun Cai","orcid":null,"position":7,"is_corresponding":false},{"id":1586972,"name":"Yege Bi","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Dysregulation of the ubiquitin-proteasome system in von Hippel-Lindau syndrome: molecular insights and clinical perspectives.","abstract":"von Hippel-Lindau (VHL) syndrome is an autosomal dominant tumor susceptibility syndrome whose pathogenesis is closely associated with dysfunction of the ubiquitin-proteasome system (UPS). When core UPS components-E1, E2, E3 enzymes, and the proteasome-malfunction, the intracellular protein homeostasis network becomes severely disrupted, thereby driving tumorigenesis. This discovery also opens a novel perspective for addressing the therapeutic challenges of VHL syndrome. This review systematically analyzes the mechanisms of abnormally expressed enzymes within the UPS in VHL syndrome and thoroughly examines the progress in therapeutic strategies targeting various UPS components. It aims to provide a theoretical foundation for understanding the molecular mechanisms of this disease and developing precision treatment approaches.","is_dataset_classified":null,"base_score":0.0,"endowment":0.0,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"41372667","pmcid":"PMC12775121","openalex_id":null,"authors":[],"funders":[{"funder_name":"the National Natural Science Foundation of China","grant_id":"82460211","title":null},{"funder_name":"Guizhou Science and Technology Cooperation support","grant_id":"[2023] general 265","title":null}],"total_grants":2,"fwci":null,"citation_percentile":null,"influential_citations":0,"citation_trend":[],"oa_status":null,"license":null,"oa_locations":[{"url":"https://europepmc.org/articles/PMC12775121","host_type":"Europe_PMC"},{"url":"https://europepmc.org/articles/PMC12775121?pdf=render","host_type":"Europe_PMC"}],"fields_of_study":[],"mesh_terms":["Humans","Proteasome Endopeptidase Complex","Ubiquitin","Von Hippel-Lindau Tumor Suppressor Protein","von Hippel-Lindau Disease"],"keywords":["Drug therapy","Deubiquitinating Enzyme","Vhl Syndrome","E3 Ubiquitination Ligase","The Ubiquitin-proteasome System"],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[{"name":"omim"}],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-02T20:42:17.442440Z","pmid":null,"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":[]}