Abstract
Heart failure and cardiovascular disease are the primary causes of mortality in patients with Hutchinson‐Gilford Progeria Syndrome (HGPS), resulting from mutations in the LMNA gene. In this study, we investigated a familial pedigree of HGPS to derive induced pluripotent stem (iPS) cells using a nonintegrating Sendai virus method from both affected and unaffected family members. The goal was to create a comprehensive robust and reliable cellar model to study the accelerated aging disease's progression. The resulting iPSCs fulfilled all pluripotency criteria, demonstrated differentiation into all three germ layers, and retained the HGPS mutation. In addition, these iPSCs exhibited key HGPS characteristics, such as shortened telomeres, abnormal Lamin A (Progerin) expression, and reduced cardiac marker levels. Thus, our approach offers a robust, reliable, and accurate representation of the disease pathology for the aging community.
| Original language | English |
|---|---|
| Pages (from-to) | 493-498 |
| Number of pages | 6 |
| Journal | Aging Medicine |
| Volume | 8 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 22 Sept 2025 |
Bibliographical note
Publisher Copyright:© 2025 The Author(s). Aging Medicine published by Beijing Hospital and John Wiley & Sons Australia, Ltd.
Keywords
- HGPS
- iPSC
- progeria
- LMNA mutation
- heart failure
- cardiovascular disease
- accelerated aging
- cardiac markers and disease modeling
- nonintegrative reprogramming
- sendai virus