Article
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice.
Proceedings of the National Academy of Sciences of the United States of America - 28 Dec 2004
Fong Loren G, Ng Jennifer K, Meta Margarita, Coté Nathan, Yang Shao H, Stewart Colin L, Sullivan Terry, Burghardt Andrew, Majumdar Sharmila, Reue Karen, Bergo Martin O, Young Stephen G
Abstract excerpt
Zmpste24 is a metalloproteinase required for the processing of prelamin A to lamin A, a structural component of the nuclear lamina. Zmpste24 deficiency results in the accumulation of prelamin A within cells, a complete loss of mature lamin A, and misshapen nuclear envelopes. Zmpste24-deficient (Zmpste24(-/-)) mice exhibit retarded growth, alopecia, micrognathia, dental abnormalities, osteolytic lesions in bones,...
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