{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T08:47:28Z","timestamp":1791276448710,"version":"4.1.0"},"reference-count":49,"publisher":"The Company of Biologists","issue":"6","license":[{"start":{"date-parts":[[2020,9,2]],"date-time":"2020-09-02T00:00:00Z","timestamp":1599004800000},"content-version":"vor","delay-in-days":3061,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/3.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,6,15]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of LMNA, producing a truncated form of prelamin A, referred to as \u201cprogerin\u201d. Here, we report the isolation of na\u00efve multipotent skin-derived precursor (SKP) cells from dermal fibroblast cultures from HGPS donors. These cells form spheres and express the neural crest marker, nestin, in addition to the multipotent markers, OCT4, Sox2, Nanog and TG30; these cells can self-renew and differentiate into smooth muscle cells (SMCs) and fibroblasts. The SMCs derived from the HGPS-SKPs accumulate nuclear progerin with increasing passages. A subset of the HGPS-na\u00efve SKPs express progerin in vitro and in situ in HGPS skin sections. This is the first in vivo evidence that progerin is produced in adult stem cells, and implies that this protein could induce stem cells exhaustion as a mechanism contributing to aging. Our study provides a basis on which to explore therapeutic applications for HGPS stem cells and opens avenues for investigating the pathogenesis of other genetic diseases.<\/jats:p>","DOI":"10.1242\/bio.20121149","type":"journal-article","created":{"date-parts":[[2012,4,19]],"date-time":"2012-04-19T03:13:40Z","timestamp":1334805220000},"page":"516-526","source":"Crossref","is-referenced-by-count":40,"title":["Na\u00efve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin <i>in vivo<\/i>"],"prefix":"10.1242","volume":"1","author":[{"given":"Vera","family":"Wenzel","sequence":"first","affiliation":[{"name":"Department of Dermatology and IMETUM, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Roedl","sequence":"additional","affiliation":[{"name":"Department of Dermatology and IMETUM, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Gabriel","sequence":"additional","affiliation":[{"name":"Department of Dermatology and IMETUM, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie B.","family":"Gordon","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Alpert Medical School of Brown University, and Hasbro Children's Hospital, Providence, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meenhard","family":"Herlyn","sequence":"additional","affiliation":[{"name":"Molecular and Cellular Oncogenesis Program, The Wistar Institute, Philadelphia, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard","family":"Schneider","sequence":"additional","affiliation":[{"name":"Luxembourg Center for Systems Biomedicine, University of Luxembourg, Luxembourg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Ring","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy, Biederstein, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karima","family":"Djabali","sequence":"additional","affiliation":[{"name":"Department of Dermatology and IMETUM, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2012,4,16]]},"reference":[{"key":"2021080923294890800_b1","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1038\/323560a0","article-title":"The nuclear lamina is a meshwork of intermediate-type filaments.","volume":"323","author":"Aebi","year":"1986","journal-title":"Nature"},{"key":"2021080923294890800_b2","doi-asserted-by":"publisher","first-page":"3940","DOI":"10.1200\/JCO.2005.11.650","article-title":"Inactivation of the lamin A\/C gene by CpG island promoter hypermethylation in hematologic malignancies, and its association with poor survival in nodal diffuse large B-cell lymphoma.","volume":"23","author":"Agrelo","year":"2005","journal-title":"J. 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Biol."},{"key":"2021080923294890800_b9","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1007\/s10038-003-0025-3","article-title":"LMNA is mutated in Hutchinson-Gilford progeria (MIM 176670) but not in Wiedemann-Rautenstrauch progeroid syndrome (MIM 264090).","volume":"48","author":"Cao","year":"2003","journal-title":"J. Hum. Genet."},{"key":"2021080923294890800_b10","doi-asserted-by":"publisher","first-page":"4949","DOI":"10.1073\/pnas.0611640104","article-title":"A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells.","volume":"104","author":"Cao","year":"2007","journal-title":"Proc. Natl. Acad. Sci. 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