{"doi":"10.1016/j.yexcr.2009.05.003","title":"Nuclear protein import is reduced in cells expressing nuclear envelopathy-causing lamin A mutants","abstract":null,"journal":"Experimental Cell Research","year":2009,"id":621143,"datarank":0.611630616585858,"base_score":4.07753744390572,"endowment":4.07753744390572,"self_citation_contribution":0.611630616585858,"citation_network_contribution":0.0,"self_endowment_contribution":0.611630616585858,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":58,"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":1603751,"name":"Tilman Kiel","orcid":null,"position":1,"is_corresponding":false},{"id":1603752,"name":"Wolfgang-M. Heupel","orcid":null,"position":2,"is_corresponding":false},{"id":1573428,"name":"Manfred Wehnert","orcid":null,"position":3,"is_corresponding":false},{"id":716964,"name":"Stefan Hübner","orcid":"0000-0001-7546-4393","position":4,"is_corresponding":false},{"id":998401,"name":"Albert Busch","orcid":"0000-0003-2374-0338","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Nuclear protein import is reduced in cells expressing nuclear envelopathy-causing lamin A mutants","abstract":"Lamins, which form the nuclear lamina, not only constitute an important determinant of nuclear architecture, but additionally play essential roles in many nuclear functions. Mutations in A-type lamins cause a wide range of human genetic disorders (laminopathies). The importance of lamin A (LaA) in the spatial arrangement of nuclear pore complexes (NPCs) prompted us to study the role of LaA mutants in nuclear protein transport. Two mutants, causing prenatal skin disease restrictive dermopathy (RD) and the premature aging disease Hutchinson Gilford progeria syndrome, were used for expression in HeLa cells to investigate their impact on the subcellular localization of NPC-associated proteins and nuclear protein import. Furthermore, dynamics of the LaA mutants within the nuclear lamina were studied. We observed affected localization of NPC-associated proteins, diminished lamina dynamics for both LaA mutants and reduced nuclear import of representative cargo molecules. Intriguingly, both LaA mutants displayed similar effects on nuclear morphology and functions, despite their differences in disease severity. Reduced nuclear protein import was also seen in RD fibroblasts and impaired lamina dynamics for the nucleoporin Nup153. Our data thus represent the first study of a direct link between LaA mutant expression and reduced nuclear protein import.","is_dataset_classified":null,"base_score":4.07753744390572,"endowment":4.07753744390572,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"19442658","pmcid":null,"openalex_id":"https://openalex.org/W2043006129","authors":[],"funders":[{"funder_name":"Deutsche Forschungsgemeinschaft","grant_id":"SFB 487","title":null},{"funder_name":"European Commission","grant_id":"018690","title":null},{"funder_name":"Deutsche Forschungsgemeinschaft","grant_id":"unidentified","title":"unidentified"},{"funder_name":"Bundesministerium für Bildung und Forschung","grant_id":"","title":null}],"total_grants":4,"fwci":2.3918,"citation_percentile":0.88687261,"influential_citations":0,"citation_trend":[{"year":2012,"count":4},{"year":2013,"count":5},{"year":2014,"count":6},{"year":2015,"count":5},{"year":2016,"count":2},{"year":2017,"count":3},{"year":2018,"count":1},{"year":2019,"count":3},{"year":2020,"count":5},{"year":2021,"count":4},{"year":2022,"count":1},{"year":2023,"count":2},{"year":2024,"count":1},{"year":2026,"count":1}],"oa_status":"closed","license":"Elsevier TDM","oa_locations":[{"url":"https://api.elsevier.com/content/article/PII:S0014482709002158?httpAccept=text/xml","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S0014482709002158?httpAccept=text/plain","host_type":"publisher"},{"url":"https://doi.org/10.1016/j.yexcr.2009.05.003","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/19442658","host_type":"repository"},{"url":"https://dx.doi.org/10.1016/j.yexcr.2009.05.003","host_type":""}],"fields_of_study":["Nuclear Structure and Function","RNA Research and Splicing","Genomics and Chromatin Dynamics","0301 basic medicine","0303 health sciences","03 medical and health sciences","Active Transport, Cell Nucleus","Cell Nucleus","Fibroblasts","Green Fluorescent Proteins","HeLa Cells","Humans","Karyopherins","Lamin Type A","Mutation","Nuclear Lamina","Nuclear Pore","Nuclear Pore Complex Proteins","Nuclear Proteins","Progeria","Protein Transport","Receptors, Cytoplasmic and Nuclear","Recombinant Fusion Proteins","Skin Diseases","Exportin 1 Protein"],"mesh_terms":["Exportin 1 Protein","Cell Nucleus","Fibroblasts","HeLa Cells","Humans","Mutation","Nuclear Proteins","Progeria","Recombinant Fusion Proteins","Skin Diseases","Receptors, Cytoplasmic and Nuclear","Protein Transport","Active Transport, Cell Nucleus","Nuclear Pore","Nuclear Pore Complex Proteins","Karyopherins","Nuclear Lamina","Lamin Type A","Green Fluorescent Proteins","Hela Cells"],"keywords":["Lamin","Nuclear lamina","Biology","Mutant","Progeria","Nucleoporin","Nuclear protein","Cell biology","Nuclear transport","Nuclear pore","Nuclear localization sequence","Cell nucleus","Nuclear membrane","Inner membrane","LMNA","Genetics","Gene","Nucleus","Transcription factor","Mitochondrion","Recombinant Fusion Proteins","Green Fluorescent Proteins","Active Transport, Cell Nucleus","Nuclear Proteins","Receptors, Cytoplasmic and Nuclear","Exportin 1 Protein","Fibroblasts","Karyopherins","Lamin Type A","Skin Diseases","Nuclear Pore Complex Proteins","Protein Transport","Mutation","Humans","HeLa Cells"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-03T13:24:06.076579Z","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":[]}