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Molecules called proteins are responsible for virtually all the processes that occur inside the cells that make up living organisms. Proteins are incredibly diverse, and many thousands of proteins with different shapes, sizes and functions can be found inside a single cell. However, all of these are initially made as a linear chain of building blocks called amino acids. In order to become functioning proteins, this chain of amino acids must be folded up into the correct 3 dimensional shape. When the folding process goes wrong, abnormally shaped proteins are produced. These can be toxic and therefore ‘quality control’ systems operate inside cells to identify and eliminate incorrectly folded proteins. Many diseases, including cystic fibrosis and Alzheimer’s, are caused by defects in protein folding and/or a failure of the cell’s quality control processes. Our research is aimed at understanding how these quality control processes work. This may one day allow us to design new ways to treat protein folding diseases.
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Mullan LA, Mularczyk EJ, Kung LH,Forouhan M, Wragg JM,Goodacre R, Bateman JF,Swanton E, Briggs MD,Boot-Handford RP
Yearbook of Paediatric Endocrinology (2018)
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