Araujo-Vilar, DavidFernández-Pombo, AntíaCobelo Gómez, SilviaCastro, Ana I.Sánchez Iglesias, Sofía2024-02-052024-02-052022Araújo-Vilar, D., Fernández-Pombo, A., Cobelo-Gómez, S. et al. Lipodystrophy-associated progeroid syndromes. Hormones 21, 555–571 (2022).http://hdl.handle.net/10347/32340This version of the article has been accepted for publication, after peer review (when applicable) and is subject to Springer Nature’s AM terms of use, but is not the Version of Record and does not reflect post-acceptance improvements, or any corrections. The Version of Record is available online at: http://dx.doi.org/10.1007/s42000-022-00386-7With the exception of HIV-associated lipodystrophy, lipodystrophy syndromes are rare conditions characterized by a lack of adipose tissue, which is not generally recovered. As a consequence, an ectopic deposition of lipids frequently occurs, which usually leads to insulin resistance, atherogenic dyslipidemia, and hepatic steatosis. These disorders include certain accelerated aging syndromes or progeroid syndromes. Even though each of them has unique clinical features, most show common clinical characteristics that affect growth, skin and appendages, adipose tissue, muscle, and bone and, in some of them, life expectancy is reduced. Although the molecular bases of these Mendelian disorders are very diverse and not well known, genomic instability is frequent as a consequence of impairment of nuclear organization, chromatin structure, and DNA repair, as well as epigenetic dysregulation and mitochondrial dysfunction. In this review, the main clinical features of the lipodystrophy-associated progeroid syndromes will be described along with their causes and pathogenic mechanisms, and an attempt will be made to identify which of López-Otín’s hallmarks of aging are present.engjournal article10.1007/s42000-022-00386-7open access