Please use this identifier to cite or link to this item:
https://hdl.handle.net/10316/114107
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Ferreira-Marques, Marisa | - |
dc.contributor.author | Carvalho, André | - |
dc.contributor.author | Franco, Ana Catarina | - |
dc.contributor.author | Leal, Ana | - |
dc.contributor.author | Botelho, Mariana | - |
dc.contributor.author | Carmo-Silva, Sara | - |
dc.contributor.author | Águas, Rodolfo | - |
dc.contributor.author | Cortes, Luísa | - |
dc.contributor.author | Lucas, Vasco | - |
dc.contributor.author | Real, Ana Carolina | - |
dc.contributor.author | López-Otín, Carlos | - |
dc.contributor.author | Nissan, Xavier | - |
dc.contributor.author | Almeida, Luís Pereira de | - |
dc.contributor.author | Cavadas, Cláudia | - |
dc.contributor.author | Aveleira, Célia A. | - |
dc.date.accessioned | 2024-03-20T11:18:27Z | - |
dc.date.available | 2024-03-20T11:18:27Z | - |
dc.date.issued | 2023-12 | - |
dc.identifier.issn | 1474-9718 | pt |
dc.identifier.issn | 1474-9726 | pt |
dc.identifier.uri | https://hdl.handle.net/10316/114107 | - |
dc.description.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. | pt |
dc.language.iso | eng | pt |
dc.publisher | Wiley-Blackwell | pt |
dc.relation | CENTRO-01- 0145- FEDER- 000012 | pt |
dc.relation | CENTRO-01- 0246- FEDER- 000010 | pt |
dc.relation | COVID/BD/152130/2021 | pt |
dc.relation | IF/00825/2015 | pt |
dc.relation | LA/P/0058/2020 | pt |
dc.relation | POCI-01- 0145- FEDER- 030167 | pt |
dc.relation | SFRH/BD/120023/2016 | pt |
dc.relation | info:eu-repo/grantAgreement/UIDB/04539/2020 | pt |
dc.rights | openAccess | pt |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | pt |
dc.subject | autophagy | pt |
dc.subject | ghrelin | pt |
dc.subject | human aging | pt |
dc.subject | progeria | pt |
dc.subject | senescence | pt |
dc.subject.mesh | Adolescent | pt |
dc.subject.mesh | Child | pt |
dc.subject.mesh | Humans | pt |
dc.subject.mesh | Mice | pt |
dc.subject.mesh | Animals | pt |
dc.subject.mesh | Ghrelin | pt |
dc.subject.mesh | Quality of Life | pt |
dc.subject.mesh | Skin | pt |
dc.subject.mesh | Lamin Type A | pt |
dc.subject.mesh | Aging | pt |
dc.subject.mesh | Progeria | pt |
dc.subject.mesh | Aging, Premature | pt |
dc.title | Ghrelin delays premature aging in Hutchinson-Gilford progeria syndrome | pt |
dc.type | article | - |
degois.publication.firstPage | e13983 | pt |
degois.publication.issue | 12 | pt |
degois.publication.title | Aging Cell | pt |
dc.peerreviewed | yes | pt |
dc.identifier.doi | 10.1111/acel.13983 | pt |
degois.publication.volume | 22 | pt |
dc.date.embargo | 2023-12-01 | * |
uc.date.periodoEmbargo | 0 | pt |
item.fulltext | Com Texto completo | - |
item.grantfulltext | open | - |
item.languageiso639-1 | en | - |
item.cerifentitytype | Publications | - |
item.openairetype | article | - |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
crisitem.project.grantno | Center for Innovative Biomedicine and Biotechnology - Associate Laboratory | - |
crisitem.project.grantno | Center for Innovative Biomedicine and Biotechnology - CIBB | - |
crisitem.author.researchunit | CNC - Center for Neuroscience and Cell Biology | - |
crisitem.author.researchunit | CNC - Center for Neuroscience and Cell Biology | - |
crisitem.author.researchunit | CIBB - Center for Innovative Biomedicine and Biotechnology | - |
crisitem.author.researchunit | CNC - Center for Neuroscience and Cell Biology | - |
crisitem.author.researchunit | CIBB - Center for Innovative Biomedicine and Biotechnology | - |
crisitem.author.orcid | 0000-0001-6730-7021 | - |
crisitem.author.orcid | 0000-0001-5831-3307 | - |
crisitem.author.orcid | 0000-0001-8020-9266 | - |
Appears in Collections: | FFUC- Artigos em Revistas Internacionais I&D CIBB - Artigos em Revistas Internacionais I&D CNC - Artigos em Revistas Internacionais |
Files in This Item:
File | Description | Size | Format | |
---|---|---|---|---|
Aging Cell - 2023 - Ferreira‐Marques - Ghrelin delays premature aging in Hutchinson‐Gilford progeria syndrome.pdf | 13.72 MB | Adobe PDF | View/Open |
Page view(s)
105
checked on Oct 16, 2024
Download(s)
44
checked on Oct 16, 2024
Google ScholarTM
Check
Altmetric
Altmetric
This item is licensed under a Creative Commons License