2013
DOI: 10.18632/aging.100550
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Abstract: Lipodystrophies, characterized by partial or complete loss of adipose tissue, have been associated with mutations in the lamin A gene. It remains unclear how lamin A mutants interfere with adipose tissue formation. Hutchinson–Gilford progeria syndrome (HGPS) presents the most severe form of lamin A-associated diseases, whose patients show a complete loss of subcutaneous fat. Using iPSCs reprogrammed from HGPS fibroblasts, we induced adipocyte formation from iPSC derived embryoid bodies or from iPSC derived mes… Show more

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Cited by 45 publications
(55 citation statements)
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References 49 publications
(73 reference statements)
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“…Using adipogenesis array, we previously reported that in HGPS adipocytes, PGC‐1α was the most severely downregulated gene among the 84 genes involved in energy metabolism (Xiong et al ., 2013). Thus, to understand how progerin causes mitochondrial defects, we first examined PGC‐1α expression in HGPS fibroblasts.…”
Section: Resultsmentioning
confidence: 99%
See 1 more Smart Citation
“…Using adipogenesis array, we previously reported that in HGPS adipocytes, PGC‐1α was the most severely downregulated gene among the 84 genes involved in energy metabolism (Xiong et al ., 2013). Thus, to understand how progerin causes mitochondrial defects, we first examined PGC‐1α expression in HGPS fibroblasts.…”
Section: Resultsmentioning
confidence: 99%
“…Recent works have highlighted the role of PGC‐1α in balancing ROS in neurodegenerative disorders and aging by controlling both the induction of mitochondrial metabolism and the removal of its ROS by‐products (Austin & St‐Pierre, 2012). Our previous study on adipocyte differentiation has identified PGC‐1α as a major target that is inhibited by progerin during adipogenesis (Xiong et al ., 2013). Consistent with those results in HGPS adipocytes, we observe a clear reduction of PGC‐1α protein in HGPS fibroblasts (Fig.…”
Section: Discussionmentioning
confidence: 99%
“…Primary skin fibroblasts carrying the classic G608G mutation (HGADFN167, HGADFN164) and a control fibroblast line (HGFDFN168) were obtained from the Progeria Research Foundation and grown under the conditions as previously described (14). These fibroblasts were transfected with retroviral mixtures of KLF4, SOX2, OCT4, and C-MYC for iPS cell induction, as reported previously (52). Two independently isolated iPS colonies from HGPS HGADFN167 and normal HGFDFN168 (father of HGADFN167), as well as another previously characterized HGPS iPS colony (HGADFN164) (52), were used in this study.…”
Section: Methodsmentioning
confidence: 99%
“…These fibroblasts were transfected with retroviral mixtures of KLF4, SOX2, OCT4, and C-MYC for iPS cell induction, as reported previously (52). Two independently isolated iPS colonies from HGPS HGADFN167 and normal HGFDFN168 (father of HGADFN167), as well as another previously characterized HGPS iPS colony (HGADFN164) (52), were used in this study. In addition, we also tested two iPS lines isolated and characterized by the Progeria Research Foundation (HGADFN167 iPS 1J and HGFDFN168 iPS 1D2).…”
Section: Methodsmentioning
confidence: 99%
“…As vantagens da ultracentrifugação não estão bem elucidadas, já que o procedimento diminui a viabilidade do vírus. Diversos autores utilizaram a ultracentrifugação (HONDA et al, 2010;RAJARAJAN et al, 2012;XIONG et al, 2013), mas em nosso laboratório, foram estabelecidas linhagens de células iPS de cães, bovinos, equinos e humanos sem o uso desta tecnologia. Não foi possível determinar a importância da ultracentrifugação lentiviral para a geração de células iPS de coelhos, pois o crescimento acelerado das ADSC foi um fator limitante para a reprogramação.…”
Section: Indução De Pluripotênciaunclassified