Fibrillin microfibrils assembly

Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and non elastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for mature elastic fibers. Microfibril arrays are also abundant in dynamic tissues that do not express elastin, such as the ciliary zonules of the eye. Mutations in fibrillin-1—the principal structural component of microfibrils—cause Marfan syndrome, a heritable disease with severe aortic, ocular, and skeletal defects. Isolated fibrillin-rich microfibrils have a complex 56 nm beads-on-a-string appearance the molecular basis of their assembly and... 

Trask, T. M., Ritty, T. M., Broekelmann, T., Tisdale, C., and Mecham, R. P. (1999). N-terminal domains of fibrillin 1 and fibrillin 2 direct the formation of homodimers A possible first step in microfibril assembly. Biochem J. 340, 693-701. 

Fibrillin-1 microfibrils are highly complex extracellular polymers, and the molecular basis of assembly is not well understood. Studies have focused on defining the role of cells in regulating assembly, the molecular interactions that drive assembly, and the role of microfibril-associated molecules in the assembly process. 

Kielty, C. M., Raghunath, M., Siracusa, L. D., Sherratt, M. J., Peters, R., Shutdeworth, C. A., and Jimenez, S. A. (1998). The Tight skin mouse Demonstradon of mutant fibrillin-1 production and assembly into abnormal microfibrils. J. Cell Biol. 140, 1159-1166. 

Reinhardt, D. P., Gambee, J. E., Ono, R. N., Bachinger, H. P., and Sakai, L. Y. (2000b). Initial steps in assembly of microfibrils. Formation of disulfide-cross-linked multi-mers containing fibrillin-1./. Biol. Chem. 275, 2205-2210. 

---