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Correlated alterations in genome organization, histone methylation, and DNA-lamin A/C interactions in Hutchinson-Gilford progeria syndrome.
TLDR
The results support a model that the accumulation of progerin in the nuclear lamina leads to altered H3K27me3 marks in heterochromatin, possibly through the down-regulation of EZH2, and disrupts heterochromeatin-lamina interactions, and trigger the global loss of spatial chromatin compartmentalization in late passage HGPS fibroblasts.
Methylene blue alleviates nuclear and mitochondrial abnormalities in progeria
TLDR
A role of mitochondrial dysfunction in developing the premature aging phenotypes in HGPS cells is demonstrated and mitochondrial‐targeting antioxidant methylene blue is suggested as a promising therapeutic approach for HGPS.
Mechanisms controlling the smooth muscle cell death in progeria via down-regulation of poly(ADP-ribose) polymerase 1
TLDR
It is shown that HGPS SMCs exhibit a profound proliferative defect, which is primarily caused by caspase-independent cell death, which demonstrates a critical role of PARP1 in mediating SMC loss in patients with HGPS and elucidates a molecular pathway underlying the progressive SMC Loss in progeria.
Lamin A and microtubules collaborate to maintain nuclear morphology
TLDR
It is found that LA knockout cells exhibit a crescent shape morphology associated with the microtubule-organizing center, which ameliorates upon treatment with MT drugs, Nocodazole or Taxol, and describes a collaborative effort between LA and the MT network to maintain nuclear morphology.
A Tissue Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome Using Human iPSC-derived Smooth Muscle Cells
TLDR
A functional three-dimensional model of HGPS is produced that replicates an arteriole-scale tissue engineered blood vessel (TEBV) using induced pluripotent stem cell (iPSC)-derived SMCs from an HGPS patient, and the ability of this iPSC-derived TEBV to reproduce key features of HG PS and respond to drugs is shown.
Mouse models of laminopathies
TLDR
This review groups these LMNA‐related mouse models into three categories: null mutants, point mutants, and progeroid mutants to compare their phenotypes and discuss their potential implications in laminopathies and aging.
Comprehensive map of age-associated splicing changes across human tissues and their contributions to age-associated diseases
TLDR
This study presents the first systematic investigation of age-associated splicing changes across tissues, and finds that genome-wide splicing profile is a better predictor of biological age than the gene and transcript expression profiles, and furthermore, age-related splicing provides additional independent contribution to age- associated complex diseases.
Loss of H3K9me3 Correlates with ATM Activation and Histone H2AX Phosphorylation Deficiencies in Hutchinson-Gilford Progeria Syndrome
TLDR
An early DDR defect of attenuated gammaH2AX signals in G0/G1 phase HGPS cells is demonstrated and a plausible connection between H3K9me3 loss and DDR deficiency is provided.
Comparing lamin proteins post-translational relative stability using a 2A peptide-based system reveals elevated resistance of progerin to cellular degradation
TLDR
The results obtained using a novel platform for inferring differences in lamin stability, based on normalization to a co-translated reporter protein following porcine teschovirus-1 2A peptide-mediated co- translational cleavage, support the notion that progerin is more stable than lamin A.
Methylene Blue Alleviates Nuclear and Mitochondrial Abnormalities in Hutchinson‐Gilford Progeria Syndrome
TLDR
A role of mitochondrial dysfunction in developing the premature aging phenotypes in HGPS cells is demonstrated and a mitochondrial‐targeting antioxidant methylene blue is suggested as a promising therapeutic for HGPS.
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