Adipose tissue dynamics in Laminopathies

Lamin A, the major splicing product of the LMNA gene, is the main constituent of the nuclear lamina, a filamentous network underneath the nuclear membrane. Lamin A, in association with its nuclear envelope partners, plays a pivotal role in the organization of the nuclear architecture and in the regulation of several nuclear processes. Mutations in nuclear lamina/nuclear envelope proteins cause rare genetic diseases collectively referred to as laminopathies. Although clinically different from the other, all laminopathies present with adipose tissue dysfunction of various severities. It is becoming increasingly evident that adipose tissue dysfunction contributes to the pathogenesis of laminopathies in multiple organs. To date, neither lipodystrophy nor lipoatrophy has been improved by any of the pharmacological approaches attempted in laminopathic patients. However, many research groups are strongly committed to understanding the molecular mechanism(s) underlying LMNA-related adipose tissue loss. The aim of this Special Issue is to collect and summarize data, which can clarify the role of prelamin A in adipose tissue dynamics and pathogenetic pathways in order to provide relevant hints to refine current therapeutic strategies and suggest more efficient therapies.