Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome

By Paola Scaffidi, Tom Misteli. Nature Medicine, 2005

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Human iPSC-based Modeling of Late-Onset Disease via Progerin-induced Aging (2013, positive): “Fibroblasts from old donors resembled HGPS fibroblasts supporting previous findings by Misteli and colleagues (Scaffidi and Misteli, 2005, 2006).”
"Laminopathies": a wide spectrum of human diseases. (2007, background): “In fibroblasts from human subjects with Hutchinson-Gilford progeria syndrome, introduction of wild type lamin A does not rescue cellular morphological abnormalities; however, correction of the aberrant splicing event using a modified oligonucleotide has been reported to reverse abnormalities in nucl”
Beyond the sequence: cellular organization of genome function. (2007, positive): “Interestingly, a hallmark of at least one of the lamin A-mediated genetic diseases is the dramatic change in histone-modification patterns and the almost complete loss of heterochromatin (Scaffidi and Misteli, 2005; Shumaker et al., 2006).”
Nuclear lamins: major factors in the structural organization and function of the nucleus and chromatin. (2008, neutral): “In addition to the changes in histone methylation, HP1 , which is normally associated with H3K9me3, was found to be down-regulated and partially dissociated from H3K9me3 sites in HGPS fibroblasts (Scaffidi and Misteli 2005; Shumaker et al. 2006).”