Post-natal myogenic and adipogenic developmental

• DOI: 10.4161/nucl.2.3.15731
• Publication Date: 2011-07-01T18:59:55Z
• Authors: Nard Kubben; Jan Willem Voncken; Gonda Konings; Michel van Weeghel; Maarten M.G. van ...; Marion Gijbels; Arie van Erk; Kees Schoonderwoerd; Bianca van den Bosch; Vivian Dahlmans; Chantal Calis; Sander M. Houten; Tom Misteli; Yigal M. Pinto
• Keywords: LMNA; Nuclear lamina; Dorsal aorta

A-type lamins are a major component of the nuclear lamina. Mutations in LMNA gene, which encodes A and C, cause set phenotypically diverse diseases collectively called laminopathies. While adult null mice show various symptoms typically associated with laminopathies, effect loss lamin A/C on early post-natal development is poorly understood. Here we developed novel mouse (LMNAGT-/-) based genetrap technology analyzed its development. We detect transcripts heart, outflow tract, dorsal aorta, liver somites during embryonic Loss results severe growth retardation developmental defects including impaired myocyte hypertrophy, skeletal muscle hypotrophy, decreased amounts subcutaneous adipose tissue ex vivo adipogenic differentiation. These death at 2 to 3 weeks post partum weakness metabolic complications, but without occurrence dilated cardiomyopathy or an obvious progeroid phenotype. Our indicate that defective critically contributes disease phenotypes