Article
ER stress-mediated apoptosis in a new mouse model of osteogenesis imperfecta.
PLoS genetics - 1 Feb 2008
Lisse Thomas S, Thiele Frank, Fuchs Helmut, Hans Wolfgang, Przemeck Gerhard K H, Abe Koichiro, Rathkolb Birgit, Quintanilla-Martinez Leticia, Hoelzlwimmer Gabriele, Helfrich Miep, Wolf Eckhard, Ralston Stuart H, Hrabé de Angelis Martin
Abstract excerpt
Osteogenesis imperfecta is an inherited disorder characterized by increased bone fragility, fractures, and osteoporosis, and most cases are caused by mutations affecting the type I collagen genes. Here, we describe a new mouse model for Osteogenesis imperfecta termed Aga2 (abnormal gait 2) that was isolated from the Munich N-ethyl-N-nitrosourea mutagenesis program and exhibited phenotypic variability, including...
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