{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Gupta MK"],"funding":["NHLBI NIH HHS","NIMH NIH HHS","U.S. Department of Health &amp; Human Services | NIH | National Heart, Lung, and Blood Institute","U.S. Department of Health &amp; Human Services | NIH | National Institute of Mental Health"],"pagination":["23"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6328609"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["10(1)"],"pubmed_abstract":["Nuclear envelopathies are recognized genetic disorders affecting individuals with mutations in their genes encoding members of the lamin family of nuclear envelope proteins that are responsible for maintaining the architectural structure of the nucleus. Irregularity in shape and size of the nuclei, nuclear membrane rupture, and appearance of micronuclei in the cytoplasm are among the pathological features of the syndrome. Here, we demonstrate that Bcl2-associated anthanogene-3 (BAG3), a stress-induced co-chaperone protein that by association with heat-shock protein 70 (HSP70) participates in regulation of autophagy, plays a critical role in the integrity of the nuclear membrane in cardiomyocytes. Cells subjected to proteotoxic stress or BAG3 downregulation show perinuclear accumulation of "],"journal":["Cell death & disease"],"pubmed_title":["Lamin B is a target for selective nuclear PQC by BAG3: implication for nuclear envelopathies."],"pmcid":["PMC6328609"],"funding_grant_id":["R01 HL123093","P30 MH092177","P30MH092177","R01HL123093"],"pubmed_authors":["Khalili K","Gupta MK","Myers VD","Feldman AM","Glauser GM","Gordon J","Cheung JY"],"additional_accession":[]},"is_claimable":false,"name":"Lamin B is a target for selective nuclear PQC by BAG3: implication for nuclear envelopathies.","description":"Nuclear envelopathies are recognized genetic disorders affecting individuals with mutations in their genes encoding members of the lamin family of nuclear envelope proteins that are responsible for maintaining the architectural structure of the nucleus. Irregularity in shape and size of the nuclei, nuclear membrane rupture, and appearance of micronuclei in the cytoplasm are among the pathological features of the syndrome. Here, we demonstrate that Bcl2-associated anthanogene-3 (BAG3), a stress-induced co-chaperone protein that by association with heat-shock protein 70 (HSP70) participates in regulation of autophagy, plays a critical role in the integrity of the nuclear membrane in cardiomyocytes. Cells subjected to proteotoxic stress or BAG3 downregulation show perinuclear accumulation of ","dates":{"release":"2019-01-01T00:00:00Z","publication":"2019 Jan","modification":"2025-04-04T10:13:39.428Z","creation":"2019-03-26T22:36:28Z"},"accession":"S-EPMC6328609","cross_references":{"pubmed":["30631036"],"doi":["10.1038/s41419-018-1255-9"]}}