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https://hdl.handle.net/10316/114107
Title: | Ghrelin delays premature aging in Hutchinson-Gilford progeria syndrome | Authors: | Ferreira-Marques, Marisa Carvalho, André Franco, Ana Catarina Leal, Ana Botelho, Mariana Carmo-Silva, Sara Águas, Rodolfo Cortes, Luísa Lucas, Vasco Real, Ana Carolina López-Otín, Carlos Nissan, Xavier Almeida, Luís Pereira de Cavadas, Cláudia Aveleira, Célia A. |
Keywords: | autophagy; ghrelin; human aging; progeria; senescence | Issue Date: | Dec-2023 | Publisher: | Wiley-Blackwell | Project: | CENTRO-01- 0145- FEDER- 000012 CENTRO-01- 0246- FEDER- 000010 COVID/BD/152130/2021 IF/00825/2015 LA/P/0058/2020 POCI-01- 0145- FEDER- 030167 SFRH/BD/120023/2016 info:eu-repo/grantAgreement/UIDB/04539/2020 |
Serial title, monograph or event: | Aging Cell | Volume: | 22 | Issue: | 12 | Abstract: | Hutchinson-Gilford progeria syndrome (HGPS) is a rare and fatal genetic condition that arises from a single nucleotide alteration in the LMNA gene, leading to the production of a defective lamin A protein known as progerin. The accumulation of progerin accelerates the onset of a dramatic premature aging phenotype in children with HGPS, characterized by low body weight, lipodystrophy, metabolic dysfunction, skin, and musculoskeletal age-related dysfunctions. In most cases, these children die of age-related cardiovascular dysfunction by their early teenage years. The absence of effective treatments for HGPS underscores the critical need to explore novel safe therapeutic strategies. In this study, we show that treatment with the hormone ghrelin increases autophagy, decreases progerin levels, and alleviates other cellular hallmarks of premature aging in human HGPS fibroblasts. Additionally, using a HGPS mouse model (LmnaG609G/G609G mice), we demonstrate that ghrelin administration effectively rescues molecular and histopathological progeroid features, prevents progressive weight loss in later stages, reverses the lipodystrophic phenotype, and extends lifespan of these short-lived mice. Therefore, our findings uncover the potential of modulating ghrelin signaling offers new treatment targets and translational approaches that may improve outcomes and enhance the quality of life for patients with HGPS and other age-related pathologies. | URI: | https://hdl.handle.net/10316/114107 | ISSN: | 1474-9718 1474-9726 |
DOI: | 10.1111/acel.13983 | Rights: | openAccess |
Appears in Collections: | FFUC- Artigos em Revistas Internacionais I&D CIBB - Artigos em Revistas Internacionais I&D CNC - Artigos em Revistas Internacionais |
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Aging Cell - 2023 - Ferreira‐Marques - Ghrelin delays premature aging in Hutchinson‐Gilford progeria syndrome.pdf | 13.72 MB | Adobe PDF | View/Open |
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