Mutation Modeling Human Laminopathies

), which causes a sparse coat and small external ears in heterozygotes and lethality in homozygotes by postnatal day 10.+ mice were hypomineralized and total body fat was deficient in males. In homozygotes, skin and oral mucosae were dysplastic and ulcerated. Nuclear morphometry of cultured cells revealed gene dose-dependent blebbing and wrinkling. mice should provide a useful new model for investigations of the pathogenesis of laminopathies