Article
Selective retention and degradation of molecules with a single mutant alpha1(I) chain in the Brtl IV mouse model of OI.
Matrix biology : journal of the International Society for Matrix Biology - 1 Oct 2007
Forlino Antonella, Kuznetsova Natalia V, Marini Joan C, Leikin Sergey
Abstract excerpt
We investigated the secretion, matrix incorporation and interactions of molecules with one and two mutant alpha1(I) collagen chains in the Brtl IV murine model for Osteogenesis Imperfecta, carrying a Gly-349 to Cys substitution in one col1a1 allele. We detected a significant deviation from the expected 25 and 50% content of the molecules with no (37-46%) and one (26-40%) mutant chains in skin and bone as well as...
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