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1.
FIGURE 1.

FIGURE 1. From: Extracellular Microfibrils in Vertebrate Development and Disease Processes.

Schematic representation of prototypical fibrillin and LTBPs (not in scale). Sites of interactions between fibrillin and TGFβ and BMP complexes are shown. For a detailed description of the structural features of fibrillins and LTBPs, see Hubmacher et al. (2). SLC, small latent complex.

Francesco Ramirez, et al. J Biol Chem. 2009 May 29;284(22):14677-14681.
2.
FIGURE 2.

FIGURE 2. From: Extracellular Microfibrils in Vertebrate Development and Disease Processes.

Diagram highlighting the main steps in microfibril biogenesis. They include the polymerization of fibrillins in a head-to-tail organization (step a) that is visualized by electron microscopy as multiple strings with regularly spaced beads (step b). The beads correspond to the N-terminal regions of fibrillins containing the Gly/Pro-rich stretch (arch) and the sites interacting with TGFβ and BMP complexes, whereas the strings correspond to the central sequence of multiple cbEGF motifs interspersed with a few TB/8-Cys modules (see Fig. 1). Also shown are microfibrils (step c) growing into large macro-aggregates that are either devoid of elastin (step d) or associated with cross-linked elastin (gray core) in elastic fibers (step e). Orange, blue, and black circles depict microfibril- and elastin-interacting molecules; additionally, some microfibrils are shown buried within amorphous elastin. A detailed description of microfibril and elastic fiber biogenesis can be found in several recent reviews (2, 4, 5).

Francesco Ramirez, et al. J Biol Chem. 2009 May 29;284(22):14677-14681.

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