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-Gilford 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 pathophysiology 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.