Back to Search
Start Over
Dissecting the Fibrillin Microfibril: Structural Insights into Organization and Function
- Source :
- Structure. 20(2):215-225
- Publication Year :
- 2012
- Publisher :
- Elsevier BV, 2012.
-
Abstract
- Force-bearing tissues such as blood vessels, lungs, and ligaments depend on the properties of elasticity and flexibility. The 10 to 12 nm diameter fibrillin microfibrils play vital roles in maintaining the structural integrity of these highly dynamic tissues and in regulating extracellular growth factors. In humans, defective microfibril function results in several diseases affecting the skin, cardiovascular, skeletal, and ocular systems. Despite the discovery of fibrillin-1 having occurred more than two decades ago, the structure and organization of fibrillin monomers within the microfibrils are still controversial. Recent structural data have revealed strategies by which fibrillin is able to maintain its architecture in dynamic tissues without compromising its ability to interact with itself and other cell matrix components. This review summarizes our current knowledge of microfibril structure, from individual fibrillin domains and the calcium-dependent tuning of pairwise interdomain interactions to microfibril dynamics, and how this relates to microfibril function in health and disease.
- Subjects :
- Models, Molecular
Fibrillin-1
Amino Acid Motifs
Biology
Fibrillins
Extracellular matrix
03 medical and health sciences
Protein structure
Fibrillin Microfibrils
Structural Biology
Animals
Humans
Protein Structure, Quaternary
Molecular Biology
030304 developmental biology
0303 health sciences
030302 biochemistry & molecular biology
Microfilament Proteins
Structural integrity
Microfilament Protein
Anatomy
Elasticity
3. Good health
Cell biology
Protein Structure, Tertiary
Microfibrils
Mutation
Calcium
Microfibril
Fibrillin
Protein Binding
Subjects
Details
- ISSN :
- 09692126
- Volume :
- 20
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Structure
- Accession number :
- edsair.doi.dedup.....f0d54524597a8d0ad739e74b0dde202a
- Full Text :
- https://doi.org/10.1016/j.str.2011.12.008