Sandbox Reserved 1645: Difference between revisions

The FBN-1 gene is involved in a variety of embryonic developmental programs. The microfibrils that are made from fibrillin-1 contribute to both elastic and non-elastic structures. The formation of the elastic fibers in the heart valves and the aorta require the involvement of both FBN-1 and FBN-2.It has been shown that both FBN-1 and FBN-2, along with the other components of elastic fibers, are expressed in the embryonic semilunar valves as early as 4 weeks of gestation. These molecules interact to form the elastic fibers in the ventricularis layer of the semilunar valves. Fibrillin-1 and fibrillin-2 are also crucial for the development of elastic fibers in the aorta. While expression of fibrillin-2 decreases significantly after fetal development, the expression of fibrillin-1 continues into adulthood. This supports the idea that fibrilin-2 dictates the development of early elastic fibers, while fibrillin-1 provides the structural support of mature elastic fibers.<ref>Quondamatteo F, Reinhardt DP, Charbonneau NL, Pophal G, Sakai LY, Herken R (December 2002). "Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development". Matrix Biology. 21 (8): 637–46. doi:10.1016/s0945-053x(02)00100-2. PMID 12524050. / Ammash NM, Sundt TM, Connolly HM (January 2008). "Marfan syndrome-diagnosis and management". Current Problems in Cardiology. 33 (1): 7–39. doi:10.1016/j.cpcardiol.2007.10.001. PMID 18155514. / Votteler M, Berrio DA, Horke A, Sabatier L, Reinhardt DP, Nsair A, Aikawa E, Schenke-Layland K (June 2013). "Elastogenesis at the onset of human cardiac valve development". Development. 140 (11): 2345–53. doi:10.1242/dev.093500. PMC 3912871. PMID 23637335.</ref>