| 16905 | P48678 | LMNA_MOUSE | Prelamin-A/C [Cleaved into: Lamin-A/C] | DISRUPTION PHENOTYPE | Mutant mice survive postnatally for 6-8 weeks and show skeletal and cardiac myopathy, sarcopenia, osteopenia, decreased bone formation, neuropathy, abnormal neuromuscular junctions, decreased skeletal muscle growth and decreased muscle satellite cell proliferation. They develop ventricular dilation and cardiac dysfunction. Within 2-3 weeks they show a reduction in their growth rate and by week 4 their growth ceases with their mean body weight being half of that of the wild-type or the heterozygous littermates. Simultaneous knockout of LMNA and LAP2 results in partial rescue of the phenotype, with a 30% increase in survival rate and a 25-50% increase in body weight. Double knockouts for MLIP and LMNA die sooner than single LMNA knockout. They develop much more severe ventricular dilation and cardiac dysfunction (PubMed:26436652). {ECO:0000269|PubMed:10579712, ECO:0000269|PubMed:11799477, ECO:0000269|PubMed:19124654, ECO:0000269|PubMed:21547077, ECO:0000269|PubMed:21982926, ECO:0000269|PubMed:23535822, ECO:0000269|PubMed:26436652}. |