Dysfunction of iPSC-derived endothelial cells in human Hutchinson–Gilford progeria syndrome

G Matrone, RA Thandavarayan, BK Walther, S Meng… - Cell Cycle, 2019 - Taylor & Francis
ABSTRACT Children with Hutchinson–Gilford progeria syndrome (HGPS) succumb to
myocardial infarction and stroke in their teen years. Endothelial dysfunction is an early event …

Extra-skeletal effects of bisphosphonates

A Panagiotakou, M Yavropoulou, N Nasiri-Ansari… - Metabolism, 2020 - Elsevier
Abstract Background Bisphosphonates (BPs) are pyrophosphate analogues widely used in
diseases related to bone loss and increased bone turnover. Their high affinity for bone …

Discovery of lonafarnib-like compounds: pharmacophore modeling and molecular dynamics studies

S Rampogu, A Baek, M Son, C Park, S Yoon… - ACS …, 2020 - ACS Publications
Progeria is a globally noticed rare genetic disorder manifested by premature aging with no
effective treatment. Under these circumstances, farnesyltransferase inhibitors (FTIs) are …

Prelamin A and ZMPSTE24 in premature and physiological aging

HJ Worman, S Michaelis - Nucleus, 2023 - Taylor & Francis
As human longevity increases, understanding the molecular mechanisms that drive aging
becomes ever more critical to promote health and prevent age-related disorders. Premature …

Isoprenylcysteine carboxylmethyltransferase-based therapy for Hutchinson–Gilford progeria syndrome

B Marcos-Ramiro, A Gil-Ordóñez… - ACS Central …, 2021 - ACS Publications
Hutchinson–Gilford progeria syndrome (HGPS, progeria) is a rare genetic disease
characterized by premature aging and death in childhood for which there were no approved …

Targeting the phospholipase A2 receptor ameliorates premature aging phenotypes

A Griveau, C Wiel, B Le Calvé, DV Ziegler… - Aging …, 2018 - Wiley Online Library
Hutchinson–Gilford progeria syndrome (HGPS) is a lethal premature aging that recapitulates
many normal aging characteristics. This disorder is caused by mutation in the LMNA gene …

[HTML][HTML] Progerin, an Aberrant Spliced Form of Lamin A, Is a Potential Therapeutic Target for HGPS

BH Kim, YH Chung, TG Woo, SM Kang, S Park, BJ Park - Cells, 2023 - mdpi.com
Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder caused
by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA …

Genomic instability and innate immune responses to self-DNA in progeria

S Gonzalo, N Coll-Bonfill - GeroScience, 2019 - Springer
In the last decade, we have seen increasing evidence of the importance of structural nuclear
proteins such as lamins in nuclear architecture and compartmentalization of genome …

Epidemiological characteristics of patients with Hutchinson-Gilford progeria syndrome and progeroid laminopathies in China

J Wang, Q Yu, X Tang, LB Gordon, J Chen, B Jiang… - Pediatric …, 2024 - nature.com
Abstract Background Hutchinson-Gilford progeria syndrome (HGPS) and progeroid
laminopathies (PL) are extremely rare genetic diseases with extremely poor prognoses. This …

[HTML][HTML] Intermittent treatment with farnesyltransferase inhibitor and sulforaphane improves cellular homeostasis in Hutchinson-Gilford progeria fibroblasts

D Gabriel, DD Shafry, LB Gordon, K Djabali - Oncotarget, 2017 - ncbi.nlm.nih.gov
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic condition associated with
mutations in the LMNA gene. This disease recapitulates some aspects of normal aging, such …