Article
Molecular basis of heritable connective tissue disease.
Biochemical medicine and metabolic biology - 1 Feb 1993
Vandenberg P
Abstract excerpt
The family of collagens represents a series of highly vulnerable gene-protein systems. This can be explained by the fact that both the folding of the pro alpha chains and the assembly of collagen monomers into fibrils highly depend on the principle of nucleated growth, in which every subunit of the system must have the correct structure. DNA analysis showed that over 90% of patients with OI have mutations in one...
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