This article basically talks about what type of diseases can occur if proteins are folded defectively or if the cell’s quality control apparatus fails. In order for proteins to properly function, all of its necessary components must be correctly compartmentalized. This article also presents ways for therapeutic intervention.
One type of disease that can occur from conformational errors is cataracts. Conformational diseases are disorders in which the structure of the fundamental protein mutates, leading to the aggregation and deposition of abnormal proteins. Cataracts is when there is clouding in the lens of your eye.
The major structural protein in the lens is crystallins, which form complex protein-protein interactions with each other. There are several types of crystallins, such as α-Crystallins, which act as molecular chaperones to prevent the aggregation and precipitation of the crystallins. β-Crystallins contain aggregates that range in size from dimers to octomers. A major lens protein is αB-Crystallin because it has a structural role in maintaining the transparency of the lens and confers protection to cells against thermal, osmotic, and oxidative damages.
αB-Crystallin's non-lenticular function is to bind irreversibly to denaturing proteins, thus preventing the formation of large light-scattering aggregates. This function is significantly important in the lens because it prevents cataracts from occurring. Proteins are prone to denaturation and aggregation and are subjected to osmotic and oxidative stresses over the lifetime of the organism.
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