Phenotype Image Detail

Image

Caption

Ultrastructure of microfibrils in P8 wild-type (+/+) and Fbn1^tm1.2Lysa/Fbn1^tm1.2Lysa (GT-8/GT-8) mice. Immunogold labeling of P8 wild-type and Fbn1^tm1.2Lysa/Fbn1^tm1.2Lysa littermate skin demonstrated differences in microfibril ultrastructure. Wild-type microfibrils with fibrillin-1 antibodies showed periodic gold labeling, which was not detectable on fibrillin-1-labeled microfibrils in homozygous skin. In contrast to wild-type microfibrils, which displayed minimal immunogold labeling, homozygous microfibrils were well labeled with fibrillin-2 antibodies. Revealing of fibrillin-2 epitopes indicated a perturbation of microfibril structure. Scale bar = 100 nm.

This image is from Charbonneau NL, J Biol Chem 2010 Aug 6;285(32):24943-55 and is displayed with the permission of the American Society for Biochemistry and Molecular Biology who owns the Copyright. Full text from JBC. J:165897

Associated
Alleles

Symbol	Name
Fbn1^tm1.2Lysa	fibrillin 1; targeted mutation 1.2, Lynn Y Sakai

Associated
Genotypes

Allelic Composition	Genetic Background
Fbn1^tm1.2Lysa/Fbn1^tm1.2Lysa	involves: C57BL/6

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