Laminopathies are tissue-specific or systemic disor-ders caused by mutations in LMNA gene encodinglamin A/C (primary laminopathies) or in genesencoding proteins with structural and/or functionalrelationship with lamin A/C (secondary laminopa-thies). Since 1994, when the first laminopathy, type 1Emery-Dreifuss Muscular Dystrophy, was associatedto the EMD gene [1] and much more since 1999,when LMNA mutations were linked to the type 2form of the same muscular dystrophy [2], the interestin lamin and nuclear envelope role in disease hasgrown exponentially. Even more because, unexpect-edly,morethantendifferentclinicalentitiesincludingdisorders of adipose tissue, cardiomyopathies, andaccelerated ageing syndromes have been linked toLMNA mutations in the following years and at leastthe same number of diseases have been associatedwith mutations in other nuclear envelope genes.This has in turn pushed forward basic research inthe field of lamin and nuclear envelope function,and international highly engaged and motivatedteams around the world have provided excitingadvances in the understanding of pathologies andtheir molecular basis.
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机译：椎板病变是组织特异性或全身性疾病，由编码lamin A / C的LMNA基因突变（原发性lamopathies）或与lamin A / C具有结构和/或功能关系的蛋白的基因编码（继发的laminpa-thies）引起。自1994年以来，当第一个椎板病（1Emery-Dreifuss肌营养不良症）与EMD基因相关[1]时，自1999年以来，LMNA突变与同一肌营养不良症的2型形式相关[2]核包膜在疾病中的作用呈指数增长。甚至更多的原因是，在接下来的几年中，包括脂肪组织疾病，心肌病和加速衰老综合征在内的多种不同的临床疾病与LMNA突变相关，并且至少相同数量的疾病与其他核被膜基因的突变相关。核纤层蛋白和核包膜功能领域的基础研究，以及国际上高度参与和积极进取的团队，在了解病理学及其分子基础方面提供了令人兴奋的进展。
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