Naı̈ve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo
نویسندگان
چکیده
Vera Wenzel*, Daniela Roedl*, Diana Gabriel*, Leslie B. Gordon, Meenhard Herlyn, Reinhard Schneider, Johannes Ring and Karima Djabali Department of Dermatology and IMETUM, Technische Universität München, Munich, Germany Department of Pediatrics, Alpert Medical School of Brown University, and Hasbro Children’s Hospital, Providence, USA Molecular and Cellular Oncogenesis Program, The Wistar Institute, Philadelphia, USA Luxembourg Center for Systems Biomedicine, University of Luxembourg, Luxembourg Department of Dermatology and Allergy, Biederstein, Technische Universität München, Munich, Germany
منابع مشابه
Naïve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo
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 "progerin". Here, we report the isolation of naïve multipotent skin-derived precursor (...
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The segmental premature aging disease Hutchinson-Gilford Progeria syndrome (HGPS) is caused by a truncated and farnesylated form of Lamin A called progerin. HGPS affects mesenchymal lineages, including the skeletal system, dermis, and vascular smooth muscle (VSMC). To understand the underlying molecular pathology of HGPS, we derived induced pluripotent stem cells (iPSCs) from HGPS dermal fibrob...
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Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare premature and accelerated aging disease caused by a de novo point mutation in LMNA encoding A-type lamins. Progerin, a truncated and toxic form of prelamin A, accumulates in HGPS cells nuclei and is a hallmark of the disease. We show that progerin is sequestered, together with other proteins (lamins B1/B2, emerin), into abnormally...
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Hutchinson-Gilford Progeria Syndrome (HGPS) is a devastating incurable premature aging disease caused by accumulation of progerin, a toxic lamin A mutant protein. HGPS patient-derived cells exhibit nuclear morphological abnormalities, altered signaling pathways, genomic instability, and premature senescence. Here we uncover new molecular mechanisms contributing to cellular decline in progeria. ...
متن کاملPluripotent stem cells for pathological modelling of Hutchinson-Gilford Progeria Syndrome (HGPS) and drug discovery
Progeria, also known as HGPS, is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children. This syndrome is typically caused by mutations in codon 608 (p.G608G) of the LMNA leading to the production of a mutated form of Lamin A precursor called progerin. In HGPS, progerin accumulates in cells causing progressive molecular defects including nuclear shape abno...
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تاریخ انتشار 2012