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MicroRNAs in hereditary and sporadic premature aging syndromes and other laminopathies

Diane Frankel 1, 2 Valerie Delecourt 2 Sophie Perrin 2 Karim Harhouri 2 Annachiara de Sandre-Giovannoli 2, 3 Nicolas Lévy 2, 4 Elise Kaspi 1, 2 Patrice Roll 1, 2
1 Laboratoire de Biologie Cellulaire [Hôpital de la Timone - APHM]
APHM - Assistance Publique - Hôpitaux de Marseille, TIMONE - Hôpital de la Timone [CHU - APHM]
3 CRB TAC - Centre de ressources biologiques Tissus ADN Cellules [Hôpital de la Timone - APHM]
Département de génétique médicale [Hôpital de la Timone - APHM], APHM - Assistance Publique - Hôpitaux de Marseille, TIMONE - Hôpital de la Timone [CHU - APHM]
Abstract : Hereditary and sporadic laminopathies are caused by mutations in genes encoding lamins, their partners, or the metalloprotease ZMPSTE24/FACE1. Depending on the clinical phenotype, they are classified as tissue-specific or systemic diseases. The latter mostly manifest with several accelerated aging features, as in Hutchinson-Gil-ford progeria syndrome (HGPS) and other progeroid syndromes. MicroRNAs are small noncoding RNAs described as powerful regulators of gene expression, mainly by degrading target mRNAs or by inhibiting their translation. In recent years, the role of these small RNAs has become an object of study in laminopathies using in vitro or in vivo murine models as well as cells/tissues of patients. To date, few miRNAs have been reported to exert protective effects in laminopathies, including miR-9, which prevents progerin accumulation in HGPS neurons. The recent literature has described the potential implication of several other miRNAs in the patho-physiology of laminopathies, mostly by exerting deleterious effects. This review provides an overview of the current knowledge of the functional relevance and molecular insights of miRNAs in laminopathies. Furthermore, we discuss how these discoveries could help to better understand these diseases at the molecular level and could pave the way toward identifying new potential therapeutic targets and strategies based on miRNA modulation.
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Submitted on : Thursday, January 10, 2019 - 12:20:48 PM
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Diane Frankel, Valerie Delecourt, Sophie Perrin, Karim Harhouri, Annachiara de Sandre-Giovannoli, et al.. MicroRNAs in hereditary and sporadic premature aging syndromes and other laminopathies. Aging Cell, Wiley Open Access, 2018, 17 (4), pp.e12766. ⟨10.1111/acel.12766⟩. ⟨hal-01925609⟩

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