5. Many connective tissue diseases result from synthesis of aberrant collagens.

Question

5. Many connective tissue diseases result from synthesis of aberrant collagens. Some of these collagens can be distinguished from native collagens by their different migratory patterns on a denaturing polyacrylamide gel (SDS-PAGE). Electrophoresis of collagen from a child who exhibits a possible collagen-deficiency disease produced a gel pattern indicative of a Type I collagen deficiency. When the separated chains were dialyzed (extracted) from the gel and recombined in an appropriate buffer, they failed to reassociate into a native triple helix. Do these data indicate that the molecular defect in the child involves the inability of collagen to form its triple helix once secreted into the extracellular matrix? Explain briefly.

Explanation / Answer

The triple native triple helix of the collagen helps in providing thermal stability, mechanical strength and its ability to interact with specific other biomolecules. Most of the damage to the collagen tissue takes place due to the substitution of the Gly residu which is involved in the ladder of the hydrogen bonds that is present in the triple helix. Due to the inability of the triple helix folding it leads to the proto-collagen chains and hence leads to the damage. So yes, the damage to the collage tissue and the molecular defect in the child is due to the inability of the collagen to form the triple helix

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