Generation of Nonintegrative‐Induced Pluripotent Stem Cells in Hutchinson‐Gilford Progeria Syndrome: Enhancing Aging Research

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.

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Publication Details

Journal
Aging Medicine
Published
2025-09-22
DOI
https://doi.org/10.1002/agm2.70041
Primary Topic
Nuclear Structure and Function
Type
article
Field-Weighted Citation Impact
0.00

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article

Generation of Nonintegrative‐Induced Pluripotent Stem Cells in Hutchinson‐Gilford Progeria Syndrome: Enhancing Aging Research

Juned Kadiwala, Rameen Shakur
Aging Medicine
Nuclear Structure and Function
article

Generation of Nonintegrative‐Induced Pluripotent Stem Cells in Hutchinson‐Gilford Progeria Syndrome: Enhancing Aging Research

Juned Kadiwala, Rameen Shakur
article en

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.

Aging MedicineVol. 8(5)
University of Brighton (GB)
University of Brighton
Openalex Percentile: Top 77%
Nuclear Structure and Function
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