fibrillin การใช้

• Fibrillin builds the oxytalan fibers, which causes the elastic behavior.
• The zonules are primarily made of fibrillin, a connective tissue protein.
• There is no complete, high-resolution structure of fibrillin-1.
• Both can be caused by mutations in the gene encoding a protein called fibrillin.
• Reducing the level of normal fibrillin 1 causes a Marfan-related disease in mice.
• Impressively, in fibrillin-1 and fibrillin-2, 43 cbEGF modules have been found.
• Impressively, in fibrillin-1 and fibrillin-2, 43 cbEGF modules have been found.
• The microfibrils that are made up of fibrillin protein are responsible for different cell-matrix interactions in the human body.
• Mutations in the fibrillin gene lead to the condition Marfan syndrome, and consequences include an increased risk of lens dislocation.
• Marfan's is a highly variable but potentially deadly disorder of connective tissue involving a defect in a protein called fibrillin.
• Fibrillin-1 is a large, extracellular matrix glycoprotein that serves as a structural component of 10-12 nm calcium-binding microfibrils.
• They have overlapping binding sites for several basement-membrane proteins, tropoelastin, fibrillin, fibronectin and proteoglycans, and they participate in diverse supramolecular structures.
• The gene normally makes a protein called fibrillin, which forms hairlike structures called microfibrils that give ligaments, tendons, artery walls and other tissues strength and resilience.
• Simply put, homocysteine is a'corrosive'of long-living proteins, i . e . collagen or elastin, or lifelong proteins, i . e . fibrillin.
• A transgenic mouse has been created carrying a single copy of a mutant fibrillin-1, a mutation similar to that found in the human gene known to cause Marfan syndrome.
• Such proteins are involved in blood coagulation or are components of the extracellular matrix like fibrillin and LTBP-1 ( Latent-transforming growth factor beta-binding protein 1 ).
• The elastic fiber is formed from the elastic microfibril ( consisting of numerous proteins such as microfibrillar-associated glycoproteins, fibrillin, fibullin, and the elastin receptor ) and amorphous elastin.
• This suggests that while the symptoms of Marfan syndrome may seem consistent with a connective tissue disorder, the mechanism is more likely related to reduced sequestration of TGF-? by fibrillin.
• Marfan's syndrome results from mutations in the " FBN1 " gene, defective production of the protein fibrillin-1, and a number of physical abnormalities including aneurysm of the aortic root.
• Truncations of the FBN1 protein in these patients were seen to have two consequences for protein production : a mutant / truncated fibrillin protein and very low plasma asprosin levels ( from a postulated dominant negative effect ).