Guillín-Amarelle, CristinaFernández-Pombo, AntíaSánchez Iglesias, SofíaAraujo-Vilar, David2024-01-312024-01-312018-04-16NUCLEUS, 2018 VOL. 9, NO. 1, 249–260 https://doi.org/10.1080/19491034.2018.14541671949-1034http://hdl.handle.net/10347/32135The nuclear lamina is a complex reticular structure that covers the inner face of the nucleus membrane in metazoan cells. It is mainly formed by intermediate filaments called lamins, and exerts essential functions to maintain the cellular viability. Lamin A/C provides mechanical steadiness to the nucleus and regulates genetic machinery. Laminopathies are tissue-specific or systemic disorders caused by variants in LMNA gene (primary laminopathies) or in other genes encoding proteins which are playing some role in prelamin A maturation or in lamin A/C function (secondary laminopathies). Those disorders in which adipose tissue is affected are called laminopathic lipodystrophies and include type 2 familial partial lipodystrophy and certain premature aging syndromes. This work summarizes the main clinical features of these syndromes, their associated comorbidities and the clues for the differential diagnosis with other lipodystrophic disorderseng© 2018 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly citedhttps://creativecommons.org/licenses/by/4.0/DiagnosisLaminopathiesLMNAProgeriaType 2 familial partial lipodystrophyjournal article10.1080/19491034.2018.14541671949-1042open access