4AK3: Crystal Structure Of Human Fibrillar Procollagen Type Iii C- Propeptide Trimer

Citation:
Abstract
The C propeptides of fibrillar procollagens have crucial roles in tissue growth and repair by controlling both the intracellular assembly of procollagen molecules and the extracellular assembly of collagen fibrils. Mutations in C propeptides are associated with several, often lethal, genetic disorders affecting bone, cartilage, blood vessels and skin. Here we report the crystal structure of a C-propeptide domain from human procollagen III. It reveals an exquisite structural mechanism of chain recognition during intracellular trimerization of the procollagen molecule. It also gives insights into why some types of collagen consist of three identical polypeptide chains, whereas others do not. Finally, the data show striking correlations between the sites of numerous disease-related mutations in different C-propeptide domains and the degree of phenotype severity. The results have broad implications for understanding genetic disorders of connective tissues and designing new therapeutic strategies.
PDB ID: 4AK3Download
MMDB ID: 102839
PDB Deposition Date: 2012/2/21
Updated in MMDB: 2012/11
Experimental Method:
x-ray diffraction
Resolution: 3.5  Å
Source Organism:
Similar Structures:
Biological Unit for 4AK3: trimeric; determined by author and by software (PISA)
Molecular Components in 4AK3
Label Count Molecule
Proteins (3 molecules)
3
Collagen Alpha-1(iii) Chain(Gene symbol: COL3A1)
Molecule annotation
Chemicals (3 molecules)
1
3
* Click molecule labels to explore molecular sequence information.

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