The main actors of this review are Hutchinson-Gilford progeria syndrome (HGPS) and atherosclerosis. HGPS is a very rare disease with no definitively approved specific drugs. Atherosclerosis is a very common disease with a more consolidated treatment strategy. Nevertheless, common mechanisms are shared by both these diseases, particularly related to inflammation, oxidative and endoplasmic reticulum (ER) stress. Pathways regulated by Nuclear factor E2 related factor (Nrf2), Nuclear factor kappa B (NF-kB) and related to the Unfolded Protein Response (UPR) and ER stress are receiving increasing attention. In HGPS "not omnia" happen(s) "cum tempore", that means that HGPS patients have atherosclerotic complications before their time. The third actor is clonal hematopoiesis: it constitutes a link between ageing and atherosclerosis. This review aims to analyse the current knowledge of atherosclerosis and clonal hematopoiesis in order to suggest therapeutic strategies to correct the timing of the atherosclerosis progression in HGPS. The goal for HGPS is a shift from "not omnia cum tempore" to "omnia cum tempore" in terms of significant lifespan extension by postponing atherosclerosis-related complications.

Progeria, atherosclerosis and clonal hematopoiesis: links and future perspectives

Mozzini, Chiara
2020-01-01

Abstract

The main actors of this review are Hutchinson-Gilford progeria syndrome (HGPS) and atherosclerosis. HGPS is a very rare disease with no definitively approved specific drugs. Atherosclerosis is a very common disease with a more consolidated treatment strategy. Nevertheless, common mechanisms are shared by both these diseases, particularly related to inflammation, oxidative and endoplasmic reticulum (ER) stress. Pathways regulated by Nuclear factor E2 related factor (Nrf2), Nuclear factor kappa B (NF-kB) and related to the Unfolded Protein Response (UPR) and ER stress are receiving increasing attention. In HGPS "not omnia" happen(s) "cum tempore", that means that HGPS patients have atherosclerotic complications before their time. The third actor is clonal hematopoiesis: it constitutes a link between ageing and atherosclerosis. This review aims to analyse the current knowledge of atherosclerosis and clonal hematopoiesis in order to suggest therapeutic strategies to correct the timing of the atherosclerosis progression in HGPS. The goal for HGPS is a shift from "not omnia cum tempore" to "omnia cum tempore" in terms of significant lifespan extension by postponing atherosclerosis-related complications.
2020
atherosclerosis
endoplasmic reticulum stress
oxidative stress
progeria
ultrasound
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/1025791
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