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首页> 外文期刊>Nucleus >Post-natal myogenic and adipogenic developmental:Defects and metabolic impairment upon loss of A-type lamins
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Post-natal myogenic and adipogenic developmental:Defects and metabolic impairment upon loss of A-type lamins

机译：产后成肌和成脂性发育：A型lamin丢失后的缺陷和代谢障碍

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摘要

A-type lamins are a major component of the nuclear lamina. Mutations in the LMNA gene, which encodes the A-type lamins A and C, cause a set of phenotypically diverse diseases collectively called laminopathies. While adult LMNA null mice show various symptoms typically associated with laminopathies, the effect of loss of lamin A/C on early post-natal development is poorly understood. Here we developed a novel LMNA null mouse (LMNA~(GT_(-/-))) based on genetrap technology and analyzed its early post-natal development. We detect LMNA transcripts in heart, the outflow tract, dorsal aorta, liver and somites during early embryonic development. Loss of A-type lamins results in severe growth retardation and developmental defects of the heart, including impaired myocyte hypertrophy, skeletal muscle hypotrophy, decreased amounts of subcutaneous adipose tissue and impaired ex vivo adipogenic differentiation. These defects cause death at 2 to 3 weeks postpartum associated with muscle weakness and metabolic complications, but without the occurrence of dilated cardiomyopathy or an obvious progeroid phenotype. Our results indicate that defective early postnatal development critically contributes to the disease phenotypes in adult laminopathies. *Correspondence
机译：A型核纤层蛋白是核纤层蛋白的主要成分。 LMNA基因中的突变编码A型核纤层蛋白A和C，导致一系列表型多样的疾病，统称为laminopathies。虽然成年LMNA无效小鼠表现出通常与椎间盘突出症相关的各种症状，但对层粘连蛋白A / C丢失对出生后早期发育的影响了解甚少。在这里，我们基于基因捕获技术开发了一种新型的LMNA null小鼠（LMNA〜（GT _（-/-））），并对其出生后的早期发育进行了分析。我们在早期胚胎发育过程中检测到心脏，流出道，背主动脉，肝脏和体节中的LMNA转录本。 A型lamin的丧失会导致严重的心脏发育迟缓和心脏发育缺陷，包括受损的心肌细胞肥大，骨骼肌萎缩，皮下脂肪组织数量减少以及离体脂肪形成分化。这些缺陷会在产后2至3周导致死亡，并伴有肌肉无力和代谢并发症，但没有发生扩张型心肌病或明显的早老表型。我们的研究结果表明，有缺陷的产后早期发育在成人椎间盘疾病中对疾病表型至关重要。 *对应

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