Complex Assemblies

1

• Extracellular Matrix Microfibrils
• Essential structural elements of matrix
• Widespread distributions in elastic and
  non-elastic tissues
• Critically important in maintaining integrity of
  tissues

• Fibrillin microfibrils
• Extensible polymers, endow connective tissues
  with long-range elasticity.
• Mutations in fibrillin-1 gene linked to Marfan
  syndrome - severe cardiovascular, ocular and
  skeletal defects.

• Complex Assemblies
• Excellent model for studying higher order
  assemblies
• Heavily cross-linked and large molecular weight
  ? cannot use traditional biochemical/structural
  techniques
• Need to develop new techniques to analyse these
  large macromolecular assemblies

2
Automated Electron Tomography
Baldock et al., (2001) J. Cell Biol. 1521045-1056
3
Pleating model of fibrillin microfibril assembly
Baldock et al., (2001) J. Cell Biol. 1521045-1056
4
Single Particle Analysis of Fibrillin Microfibrils
5
Small Angle X-ray Scattering
High Brilliance Synchrotron Solution scattering
D e t e c t o r
Protein solution
X-rays
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7
The Supramolecular organization of collagen VI
microfibrils
Baldock et al., (2003) J. Mol. Biol. 330297-307