STRUCTURAL BIOLOGY: The Heart of the Fiber

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Science  16 Mar 2001:
Vol. 291, Issue 5511, pp. 2049c
DOI: 10.1126/science.291.5511.2049c

Fibrillin is a large, multi-domain protein that contributes to the integrity of connective tissue components; skeletal and cardiovascular abnormalities in Marfan syndrome have been linked to mutations in this protein. Baldock et al. have collected data from automated electron tomography, localization of antibodies and colloidal gold, and an examination of stretched and untensioned samples. They now present a detailed proposal for how eight fibrillin monomers are packed, as dimers, into a microfibril that has been characterized as “beads-on-a-string” with a bead periodicity of 56 nanometers. Each monomer folds back on itself into segments joined by noncovalent and covalent (transglutaminase) interactions that are thought to underlie reversible extension and retraction of the extracellular matrix. — GJC

Acknowledgments

J. Cell Biol. 152, 1045 (2001).

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