Proteins are essential for life, performing many critical functions. However, proteins are 3-dimensional structures, and to be functional, they must be configured or folded correctly. If folded incorrectly, they lose their ability to interact with other molecules properly and may build up. This buildup or accumulation of misfolded proteins can contribute to diseases like retinitis pigmentosa and other conditions like Alzheimer’s. A recent study from UC Santa Barbara found a link between a protein called ZIP7 and the cell's ability to remove improperly folded proteins. ZIP7 helps transport zinc, which is vital for breaking down these faulty proteins. Researchers believe that boosting ZIP7 activity could help treat diseases caused by protein misfolding, including retinitis pigmentosa, and research is underway to test this theory in lab-grown human retinal cells.
What this means for Usher syndrome: The discovery that ZIP7 can prevent retinal degeneration opens an avenue for scientists to explore in the search for potential treatments for vision loss in Usher syndrome.