Progerinin, an Inhibitor of Progerin, Alleviates Cardiac Abnormalities in a Model Mouse of Hutchinson–Gilford Progeria Syndrome

So Mi Kang, Seungwoon Seo, Eun Ju Song, Okhee Kweon, Ah Hyeon Jo, Soyoung Park, Tae Gyun Woo, Bae Hoon Kim, Goo Taeg Oh, Bum Joon Park

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Hutchinson–Gilford Progeria Syndrome (HGPS) is an ultra-rare human premature aging disorder that precipitates death because of cardiac disease. Almost all cases of HGPS are caused by aberrant splicing of the LMNA gene that results in the production of a mutant Lamin A protein termed progerin. In our previous study, treatment with Progerinin has been shown to reduce progerin expression and improve aging phenotypes in vitro and in vivo HGPS models. In this record, cardiac parameters (stroke volume (SV), ejection fraction (EF), fractional shortening (FS), etc.) were acquired in LmnaWT/WT and LmnaG609G/WT mice fed with either a vehicle diet or a Progerinin diet by echocardiography (from 38 weeks to 50 weeks at various ages), and then the cardiac function was analyzed. We also acquired the tissue samples and blood serum of LmnaWT/WT and LmnaG609G/WT mice for pathological analysis at the end of echocardiography. From these data, we suggest that the administration of Progerinin in the HGPS model mouse can restore cardiac function and correct arterial abnormalities. These observations provide encouraging evidence for the efficacy of Progerinin for cardiac dysfunction in HGPS.

Original languageEnglish
Article number1232
JournalCells
Volume12
Issue number9
DOIs
StatePublished - May 2023

Bibliographical note

Funding Information:
This research was supported by National Research Foundation of Korea (NRF) grant funded by the Korea government (MSIT) (NRF-2020R1A4A1019322 to B.J.P.; and NRF-2020R1F1A1075370 to B.J.P.), 2023 Regional Industry-linked University Open-Lab Development Support Program through the Commercializations Promotion Agency for R&D Outcomes (COMPA) funded by Ministry of Science and ICT (2023openlab(RnD)_02), and the Progeria Research Foundation (Grant #PRF 2019-75 to B.J.P.).

Publisher Copyright:
© 2023 by the authors.

Keywords

  • cardiac dysfunction
  • fibrosis
  • Hutchinson–Gilford Progeria Syndrome (HGPS)
  • Lmna model mouse
  • progerin
  • Progerinin

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