{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T18:05:03Z","timestamp":1777572303987,"version":"3.51.4"},"update-to":[{"DOI":"10.1371\/journal.pgen.1010710","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2023,4,27]],"date-time":"2023-04-27T00:00:00Z","timestamp":1682553600000}}],"reference-count":132,"publisher":"Public Library of Science (PLoS)","issue":"4","license":[{"start":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T00:00:00Z","timestamp":1681689600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","award":["1R21HD108695"],"award-info":[{"award-number":["1R21HD108695"]}],"id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002889","name":"Foundation for Prader-Willi Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002889","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Storr Family Foundation through PWSA"},{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"publisher","award":["8P41 GM103533"],"award-info":[{"award-number":["8P41 GM103533"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosgenetics.org"],"crossmark-restriction":false},"short-container-title":["PLoS Genet"],"abstract":"<jats:p>Prader-Willi syndrome (PWS) is a multisystem disorder with neurobehavioral, metabolic, and hormonal phenotypes, caused by loss of expression of a paternally-expressed imprinted gene cluster. Prior evidence from a PWS mouse model identified abnormal pancreatic islet development with retention of aged insulin and deficient insulin secretion. To determine the collective roles of PWS genes in \u03b2-cell biology, we used genome-editing to generate isogenic, clonal INS-1 insulinoma lines having 3.16 Mb deletions of the silent, maternal- (control) and active, paternal-allele (PWS). PWS \u03b2-cells demonstrated a significant cell autonomous reduction in basal and glucose-stimulated insulin secretion. Further, proteomic analyses revealed reduced levels of cellular and secreted hormones, including all insulin peptides and amylin, concomitant with reduction of at least ten endoplasmic reticulum (ER) chaperones, including GRP78 and GRP94. Critically, differentially expressed genes identified by whole transcriptome studies included reductions in levels of mRNAs encoding these secreted peptides and the group of ER chaperones. In contrast to the dosage compensation previously seen for ER chaperones in<jats:italic>Grp78<\/jats:italic>or<jats:italic>Grp94<\/jats:italic>gene knockouts or knockdown, compensation is precluded by the stress-independent deficiency of ER chaperones in PWS \u03b2-cells. Consistent with reduced ER chaperones levels, PWS INS-1 \u03b2-cells are more sensitive to ER stress, leading to earlier activation of all three arms of the unfolded protein response. Combined, the findings suggest that a chronic shortage of ER chaperones in PWS \u03b2-cells leads to a deficiency of protein folding and\/or delay in ER transit of insulin and other cargo. In summary, our results illuminate the pathophysiological basis of pancreatic \u03b2-cell hormone deficits in PWS, with evolutionary implications for the multigenic PWS-domain, and indicate that PWS-imprinted genes coordinate concerted regulation of ER chaperone biosynthesis and \u03b2-cell secretory pathway function.<\/jats:p>","DOI":"10.1371\/journal.pgen.1010710","type":"journal-article","created":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T17:36:04Z","timestamp":1681752964000},"page":"e1010710","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":4,"title":["Insulin secretion deficits in a Prader-Willi syndrome \u03b2-cell model are associated with a concerted downregulation of multiple endoplasmic reticulum chaperones"],"prefix":"10.1371","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4175-7777","authenticated-orcid":true,"given":"Erik 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Skelin","year":"2010","journal-title":"ALTEX"},{"issue":"7","key":"pgen.1010710.ref042","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1034\/j.1600-0854.2002.30703.x","article-title":"Imaging secretory vesicles by fluorescent protein insertion in propeptide rather than mature secreted peptide","volume":"3","author":"S Watkins","year":"2002","journal-title":"Traffic"},{"issue":"23","key":"pgen.1010710.ref043","doi-asserted-by":"crossref","first-page":"4577","DOI":"10.1093\/nar\/27.23.4577","article-title":"Concerted regulation and molecular evolution of the duplicated SNRPB\u2019\/B and SNRPN loci","volume":"27","author":"TA Gray","year":"1999","journal-title":"Nucleic Acids Res"},{"issue":"4","key":"pgen.1010710.ref044","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1007\/s10735-013-9555-y","article-title":"Neural-specific expression of miR-344-3p during mouse embryonic development","volume":"45","author":"Q Liu","year":"2014","journal-title":"J Mol 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Teodoro","year":"2012","journal-title":"Am J Physiol Cell Physiol"},{"key":"pgen.1010710.ref077","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/978-1-0716-0385-7_4","article-title":"A review of mouse models of monogenic fiabetes and ER stress signaling","volume":"2128","author":"P Salpea","year":"2020","journal-title":"Methods Mol Biol"},{"key":"pgen.1010710.ref078","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1186\/1471-2121-11-59","article-title":"Endoplasmic reticulum stress response in an INS-1 pancreatic beta-cell line with inducible expression of a folding-deficient proinsulin","volume":"11","author":"T Hartley","year":"2010","journal-title":"BMC Cell Biol"},{"issue":"4","key":"pgen.1010710.ref079","doi-asserted-by":"crossref","first-page":"a033894","DOI":"10.1101\/cshperspect.a033894","article-title":"Early events in the endoplasmic reticulum unfolded protein response.","volume":"11","author":"S Preissler","year":"2019","journal-title":"Cold Spring 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Adv."},{"issue":"4","key":"pgen.1010710.ref095","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/01.mpa.0000179730.69081.64","article-title":"Gene silencing in the endocrine pancreas mediated by short-interfering RNA","volume":"31","author":"SP Bradley","year":"2005","journal-title":"Pancreas"},{"issue":"1","key":"pgen.1010710.ref096","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.cmet.2015.10.016","article-title":"Reduced insulin production relieves endoplasmic reticulum stress and induces \u03b2 cell proliferation","volume":"23","author":"M Szabat","year":"2016","journal-title":"Cell Metab"},{"issue":"2","key":"pgen.1010710.ref097","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/j.molcel.2012.07.033","article-title":"Long noncoding RNAs with snoRNA ends","volume":"48","author":"QF Yin","year":"2012","journal-title":"Mol 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Aklan","year":"2020","journal-title":"Cell Metab"},{"issue":"11","key":"pgen.1010710.ref108","doi-asserted-by":"crossref","first-page":"2281","DOI":"10.1038\/nprot.2013.143","article-title":"Genome engineering using the CRISPR-Cas9 system.","volume":"8","author":"FA Ran","year":"2013","journal-title":"Nat Protoc."},{"issue":"1","key":"pgen.1010710.ref109","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1002\/gcc.22115","article-title":"Upregulation of the ATR-CHEK1 pathway in oral squamous cell carcinomas","volume":"53","author":"RA Parikh","year":"2014","journal-title":"Genes Chromosomes Cancer"},{"issue":"8","key":"pgen.1010710.ref110","doi-asserted-by":"crossref","first-page":"e2876","DOI":"10.1371\/journal.pone.0002876","article-title":"Mathematical analysis of copy number variation in a DNA sample using digital PCR on a nanofluidic device","volume":"3","author":"S Dube","year":"2008","journal-title":"PLoS 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Krueger","year":"2015","journal-title":"\/"},{"issue":"1","key":"pgen.1010710.ref117","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1093\/bioinformatics\/bts635","article-title":"STAR: ultrafast universal RNA-seq aligner","volume":"29","author":"A Dobin","year":"2013","journal-title":"Bioinformatics"},{"issue":"2","key":"pgen.1010710.ref118","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1093\/bioinformatics\/btu638","article-title":"HTSeq\u2014a Python framework to work with high-throughput sequencing data","volume":"31","author":"S Anders","year":"2015","journal-title":"Bioinformatics"},{"issue":"12","key":"pgen.1010710.ref119","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1186\/s13059-014-0550-8","article-title":"Moderated estimation of fold change and dispersion for RNA-seq data with DESeq2","volume":"15","author":"MI Love","year":"2014","journal-title":"Genome Biol"},{"key":"pgen.1010710.ref120","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1186\/1471-2105-12-323","article-title":"RSEM: accurate transcript quantification from RNA-Seq data with or without a reference genome","volume":"12","author":"B Li","year":"2011","journal-title":"BMC Bioinformatics"},{"issue":"5","key":"pgen.1010710.ref121","doi-asserted-by":"crossref","first-page":"P3","DOI":"10.1186\/gb-2003-4-5-p3","article-title":"DAVID: Database for Annotation, Visualization, and Integrated Discovery","volume":"4","author":"G Dennis","year":"2003","journal-title":"Genome Biol"},{"key":"pgen.1010710.ref122","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1186\/1471-2105-14-128","article-title":"Enrichr: interactive and collaborative HTML5 gene list enrichment analysis tool","volume":"14","author":"EY Chen","year":"2013","journal-title":"BMC Bioinformatics"},{"issue":"3","key":"pgen.1010710.ref123","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1101\/gr.209601.116","article-title":"UMI-tools: modeling sequencing errors in Unique Molecular Identifiers to improve quantification accuracy","volume":"27","author":"T Smith","year":"2017","journal-title":"Genome Res"},{"issue":"1","key":"pgen.1010710.ref124","first-page":"3","article-title":"Cutadapt removes adapter sequences from high-throughput sequencing reads.","volume":"17","author":"M. 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