Article
New insights into the structure, assembly and biological roles of 10-12 nm connective tissue microfibrils from fibrillin-1 studies.
The Biochemical journal - 1 Apr 2016
Jensen Sacha A, Handford Penny A
Abstract excerpt
The 10-12 nm diameter microfibrils of the extracellular matrix (ECM) impart both structural and regulatory properties to load-bearing connective tissues. The main protein component is the calcium-dependent glycoprotein fibrillin, which assembles into microfibrils at the cell surface in a highly regulated process involving specific proteolysis, multimerization and glycosaminoglycan interactions. In higher...
Topics
- Animals
- Connective Tissue
- Dwarfism
- Extracellular Matrix
- Fibrillin-1
- Fibrillins
- Humans
- Marfan Syndrome
- Microfibrils
- Microfilament Proteins
- Mutation
- Osteochondrodysplasias
- Transforming Growth Factor beta
