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TranscriptomicsRecapitulation of human premature aging by using iPSCs from Hutchinson-Gilford progeria syndrome
Hutchinson-Gilford progeria syndrome (HGPS) is a rare and fatal human premature aging disease1-5, characterized by premature atherosclerosis and degeneration of vascular smooth muscle cells (SMCs)6-8. HGPS is caused by a single-point mutation in the LMNA gene, resulting in the generation of progerin...
ORGANISM(S): Homo sapiens 
2011-03-14 | E-GEOD-24487 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human Hutchinson-Gilord progeria syndrome fibroblast cell lines with dominant mutation in the LMNA gene and age-matched normal controls
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease with widespread phenotypic features resembling premature aging. HGPS was recently shown to be caused by dominant mutations in the LMNA gene, resulting in the in-frame deletion of 50 amino acids near the carboxyl terminus of the en...
ORGANISM(S): Homo sapiens 
2007-12-12 | E-GEOD-3860 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of human fibroblasts from patients with HGPS compared to age matched controls treated both control and HGPS fibroblasts with a protein farnesyltransferase inhibitor (FTI)
We analyzed and compared global gene expression changes in fibroblasts from human subjects with HGPS compared to age matched controls. We then treated both control and HGPS fibroblasts with a protein farnesyltransferase inhibitor (FTI), a medication currently used in clinical trials to treat HGPS, ...
ORGANISM(S): Homo sapiens 
2010-06-01 | E-MEXP-2597 | biostudies-arrayexpress
TranscriptomicsRNA seq of HGPS treated cells
Evaluation of transcript levels in HGPS cells upon treatment with proteasome inhibitor
ORGANISM(S): Homo sapiens 
2017-06-18 | E-MTAB-5807 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of primary fibroblasts from a family with autosomal recessive Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford progeria syndrome (HGPS) is a genetic disorder displaying features reminiscent of premature senescence caused by germline mutations in the LMNA gene encoding lamin A and C, essential components of the nuclear lamina. By studying a family with homozygous LMNA mutation (K542N), we s...
ORGANISM(S): Homo sapiens 
2011-06-27 | E-MEXP-3097 | biostudies-arrayexpress
GenomicsRNA seq of HGPS treated cells
RNA seq of HGPS treated cells
PRJEB21213 | ENA
GenomicsCorrelated alterations in genome organization, histone methylation, and DNA-lamina interactions in Hutchinson-Gilford progeria syndrome (ChIP-seq)
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease that is frequently caused by a de novo point mutation at position 1824 in LMNA. This mutation activates a cryptic splice donor site in exon 11, and leads to an in-frame deletion within the prelamin A mRNA and the production of ...
ORGANISM(S): Homo sapiens 
2012-10-30 | E-GEOD-41757 | biostudies-arrayexpress
GenomicsCorrelated alterations in genome organization, histone methylation, and DNA-lamina interactions in Hutchinson-Gilford progeria syndrome (Hi-C)
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease that is frequently caused by a de novo point mutation at position 1824 in LMNA. This mutation activates a cryptic splice donor site in exon 11, and leads to an in-frame deletion within the prelamin A mRNA and the production of ...
ORGANISM(S): Homo sapiens 
2012-10-30 | E-GEOD-41763 | biostudies-arrayexpress
ProteomicsQuantitative Proteomics Study (iTRAQ) of Progeria Cell Lines
A quantitative proteomic study of three HGPS cell lines from affected patients and three healthy controls from unaffected progenitors
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2017-10-03 | MSV000081576 | MassIVE
TranscriptomicsExpression data from transformed WT and HGPS cell lines, including HGPS cells after knock-down of BRD4
Primary skin fibroblasts from a HGPS patient and an age-matched control wild-type individual were challenged in a standard transformation assay by retroviral introduction of TERT (T), V12-HRAS (R) and SV40 large and small T antigens (S). Knock-down of BRD4 in this TRS-HGPS cell line (TRS-HGPS-shBRD4...
ORGANISM(S): Homo sapiens 
2014-10-02 | E-GEOD-60519 | biostudies-arrayexpress
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