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Understanding the transcriptional consequences of the Progeria LmnaG609G mutation within the immune compartment of the lung using single cell RNA sequencing.

ABSTRACT: To understand the transcriptional consequences of Progeria LmnaG609G mutation within the immune compartment of the lung we conducted single cell RNA sequencing on sorted immune cells from WT vs. LmnaG609G/G609G mice (in duplicate, 2 mice per group).

ORGANISM(S): Mus musculus

PROVIDER: GSE183249 | GEO | 2024/08/01

REPOSITORIES: GEO

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Understanding the transcriptional consequences of the Progeria LmnaG609G mutation within the immune compartment of the lung using single cell RNA sequencing.

Project description:Understanding the transcriptional consequences of the Progeria LmnaG609G mutation within the immune compartment of the lung using single cell RNA sequencing.

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Understanding the transcriptional consequences of Lamin A deficiency within the immune compartment of the lung using single cell RNA sequencing.

Project description:To understand the transcriptional consequences of Lamin A deficiency within the immune compartment of the lung we conducted single cell RNA sequencing on sorted immune cells from WT vs. Lmna-flox/flox Vav1-cre-/- mice (in duplicate, 2 mice per group).

2023-07-24 | GSE146080 | GEO

Bulk RNA-seq of multiple organs in LmnaG609G progeria mice with inducible Δ133p53α

Project description:Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging disorder caused by LMNA mutations that produce progerin. To investigate whether the senescence-inhibitory human p53 isoform Δ133p53α counteracts progeroid phenotypes in vivo, we generated mice carrying a Cre/loxP-inducible Δ133p53α transgene and crossed them with LmnaG609G mice. Bulk RNA-seq was performed on heart, kidney, liver, and skeletal muscle from wild-type and LmnaG609G/+ mice across five experimental groups. Transcriptomes were analyzed to identify Δ133p53α-associated gene expression programs and pathway-level changes across organs. This dataset provides a multi-organ resource to interrogate transcriptional signatures linked to Δ133p53α induction in a progeria model and support mechanistic interpretation of the associated manuscript.

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Arterial stiffness and cardiac dysfunction in Hutchinson-Gilford Progeria Syndrome corrected by inhibition of Lysyl Oxidase

Project description:Purpose: Arterial stiffening is a hallmark of premature aging in Hutchinson-Gilford Progeria Syndrome (HGPS), but the molecular regulators remain unknown. Here, we show that the LMNAG609G mouse model of HGPS recapitulates the premature arterial stiffening seen in human HGPS. To gain a better understanding of potential stiffness-regulators in LMNAG609G mice, we performed RNA-sequencing analysis on cleaned descending aortas from 2- and 24-month WT and 2-month LMNAG609G mice on a C57BL6 background. Methods: Descending aortas containing the intimal, medial and adventitial layers were isolated from 2- and 24-month male WT and 2-month HGPS mice, and RNA was extracted using the RNeasy Plus Micro kit (Qiagen 74034). The high-throughput library was prepared using the TruSeq stranded total RNA (ribo-Zero) kit (Illumina 20037135). Paired-end sequencing was performed on a HiSeq4000 Sequencing System (Illumina) and generated 14-30 million reads/sample.

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Endothelial YAP/TAZ activation is a key mediator of atherosclerosis in Hutchinson-Gilford progeria syndrome

Project description:Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare disease caused by the expression of progerin, an aberrant protein produced by a de novo point mutation in the LMNA gene. HGPS patients show accelerated aging and die prematurely, mainly from atherosclerosis complications. Understanding vascular disease onset and progression in HGPS and uncovering new therapeutic targets critically depend on the identification of cell type-specific molecular and functional alterations in the highly heterogeneous cell subsets present in the arterial wall. We used single-cell RNA sequencing to characterize the cellular and molecular landscape of the aorta in progerin-expressing LmnaG609G/G609G mice and wild type controls. Subendothelial extracellular matrix (ECM) stiffness was analyzed in decellularized aortas by atomic force microscopy, and aortic blood flow in vivo was monitored by ultrasound assessment. For atherosclerosis studies, we used progeroid atheroprone Apoe–/– LmnaG609G/G609G mice.

2024-10-17 | E-MTAB-13678 | biostudies-arrayexpress

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Project description:Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare lethal genetic disorder characterized with symptoms reminiscent of accelerated aging. The major underlying genetic cause is a substitution mutation in the gene coding for lamin A, causing the production of a toxic isoform called progerin. Here we show the RNA sequencing analyses performed on primary mouse fibroblasts of different genotypes (in terms of Progeria; Cas9 background) treated with Cas9 guide RNAs targeting Lmna/Progerin. The mouse genetic background in (1) Pro/Pro;Cas9/+: homozygous for Progeria and heterozygous for transgenic Cas9 The mouse genetic background in (2) Pro/+; +/+: heterozygous for Progeria and no transgenic Cas9 (wild type) The mouse genetic background in (3) +/+;Cas9/+: no Progeria (wild type) and heterozygous for transgenic Cas9

2019-02-15 | GSE122865 | GEO

Project description:This SuperSeries is composed of the following subset Series: GSE41751: Correlated alterations in genome organization, histone methylation, and DNA-lamina interactions in Hutchinson-Gilford progeria syndrome (expression) GSE41757: Correlated alterations in genome organization, histone methylation, and DNA-lamina interactions in Hutchinson-Gilford progeria syndrome (ChIP-seq) GSE41763: Correlated alterations in genome organization, histone methylation, and DNA-lamina interactions in Hutchinson-Gilford progeria syndrome (Hi-C) Refer to individual Series

2012-10-30 | E-GEOD-41764 | biostudies-arrayexpress

Lung endothelial cells (MLECs) from knock-in mouse model with or without the causative HGPS LmnaG609G mutation

Project description:Vascular dysfunction is one of the typical characteristics of aging, but its contributing roles to systemic aging and the therapeutic potential is lacking experimental evidence. Accumulating data suggest that the mechanisms underlying aging are similar to those governing Hutchinson-Gilford progeria syndrome (HGPS), a premature aging disease, in which affected patients succumb to cardiovascular diseases (CVDs). Here, we generated a knock-in mouse model with the causative HGPS LmnaG609G mutation, called progerin. We crossed Lmnaf/f mice with a Tie2-Cre line to get Lmnaf/f;TC mice, which exhibit defective microvasculature and neovascularization, accelerated aging and shortened lifespan. Single-cell transcriptomic analysis of murine lung endothelial cells (MLECs) revealed a significant upregulation of inflammatory response. These data support endothelial dysfunction as a primary trigger of systemic aging and highlight gene therapy as a potential strategy for the clinical treatment of HGPS and age-related vascular dysfunction.

2019-10-17 | GSE138975 | GEO

High throughput sequencing of atypical progeria caused by LMNA R527C mutation

Project description:High throughput sequencing of atypical progeria caused by LMNA R527C mutation

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Endothelial-to-mesenchymal transition contributes to accelerated atherosclerosis in Hutchinson–Gilford progeria syndrome

Project description:The main goal of this study was to gain insight into the mechanisms underlying phenotypic changes in endothelial cells during atherosclerosis associated with Hutchinson-Gilford progeria syndrome, a premature aging syndrome. To this end, we performed an RNAseq experiment with aortic intima samples from atheroprone mice with ubiquitous and VSMC-specific progeria expression and their corresponding controls.

2024-06-05 | GSE269166 | GEO

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