Premature Aging Syndromes: From Patients to Mechanism

October 2019 in “ Journal of dermatological science ”

Mattheus Xing Rong Foo, P.P. Ong, Oliver Dreesen

Hutchinson-Gilford Progeria Syndrome HGPS progerin farnesyltransferase inhibitors FTIs statins aminobisphosphonates mTOR inhibitors rapamycin Progeria

Studysummary This review explores how cell and mouse models have contributed to understanding the mechanisms of human aging, particularly focusing on Hutchinson-Gilford Progeria Syndrome, and reports no new clinical results. Our plain-language summary of this paper — not a Tressless recommendation.

The document discussed premature aging syndromes, particularly Hutchinson-Gilford Progeria Syndrome (HGPS), caused by mutations in the nuclear lamina. It highlighted that the mutant protein progerin led to premature cellular senescence, abnormal nuclear architecture, DNA damage, and loss of heterochromatin. Treatments like farnesyltransferase inhibitors (FTIs) showed limited success, while combined treatments with statins, aminobisphosphonates, and mTOR inhibitors like rapamycin showed more promise in mouse models. The study emphasized the need for further research to understand the link between progerin expression, chromatin changes, and DNA damage, and to develop effective anti-aging therapies.

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research Targeted Transgenic Expression of the Mutation Causing Hutchinson-Gilford Progeria Syndrome Leads to Proliferative and Degenerative Epidermal Disease

85 citations , March 2008 in “Journal of Cell Science”

This study created transgenic mouse models with the LMNA gene mutation common in Hutchinson-Gilford progeria syndrome, revealing skin and teeth abnormalities related to transgene expression levels.