Progeria: Translational Insights from Cell Biology

Overview

Journal J Cell Biol

Specialty Cell Biology

Date 2012 Oct 3

PMID 23027899

Citations 26

Authors

Leslie B Gordon

Kan Cao

Francis S Collins

Affiliations

Soon will be listed here.

Abstract

Cell biologists love to think outside the box, pursuing many surprising twists and unexpected turns in their quest to unravel the mysteries of how cells work. But can cell biologists think outside the bench? We are certain that they can, and clearly some already do. To encourage more cell biologists to venture into the realm of translational research on a regular basis, we would like to share a handful of the many lessons that we have learned in our effort to develop experimental treatments for Hutchinson-Gilford progeria syndrome (HGPS), an endeavor that many view as a "poster child" for how basic cell biology can be translated to the clinic.

Citing Articles

The farnesyl transferase inhibitor (FTI) lonafarnib improves nuclear morphology in ZMPSTE24-deficient fibroblasts from patients with the progeroid disorder MAD-B.

Odinammadu K, Shilagardi K, Tuminelli K, Judge D, Gordon L, Michaelis S Nucleus. 2023; 14(1):2288476.

PMID: 38050983 PMC: 10730222. DOI: 10.1080/19491034.2023.2288476.

Crystal structure of progeria mutant S143F lamin A/C reveals increased hydrophobicity driving nuclear deformation.

Ahn J, Jeong S, Kang S, Jo I, Park B, Ha N Commun Biol. 2022; 5(1):267.

PMID: 35338226 PMC: 8956589. DOI: 10.1038/s42003-022-03212-3.

Splicing Variants, Protein-Protein Interactions, and Drug Targeting in Hutchinson-Gilford Progeria Syndrome and Small Cell Lung Cancer.

Kim B, Woo T, Kang S, Park S, Park B Genes (Basel). 2022; 13(2).

PMID: 35205210 PMC: 8871687. DOI: 10.3390/genes13020165.

More Than Skin Deep - the Effects of Ultraviolet Radiation on Cathepsin K and Progerin Expression in Cultured Dermal Fibroblasts.

Bednarski I, Ciazynska M, Kabzinski J, Majsterek I, Sobolewska-Sztychny D, Narbutt J Clin Cosmet Investig Dermatol. 2021; 14:1561-1568.

PMID: 34737595 PMC: 8558101. DOI: 10.2147/CCID.S318707.

Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome.

Kang S, Yoon M, Ahn J, Kim J, Kim S, Kang S Commun Biol. 2021; 4(1):5.

PMID: 33398110 PMC: 7782499. DOI: 10.1038/s42003-020-01540-w.

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