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Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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

ABSTRACT: 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.

INSTRUMENT(S): Illumina HiSeq 2500

ORGANISM(S): Mus musculus

SUBMITTER: Carlos Torroja

PROVIDER: E-MTAB-13678 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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