Dysfunction of iPSC-derived endothelial cells in human Hutchinson–Gilford progeria syndrome
ABSTRACT Children with Hutchinson–Gilford progeria syndrome (HGPS) succumb to
myocardial infarction and stroke in their teen years. Endothelial dysfunction is an early event …
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 …
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 …
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 …
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 …
characterized by premature aging and death in childhood for which there were no approved …
Targeting the phospholipase A2 receptor ameliorates premature aging phenotypes
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 …
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 …
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 …
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 …
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 …
mutations in the LMNA gene. This disease recapitulates some aspects of normal aging, such …