Flawed quality control in the brain
New mouse type reveals when neurons fail to cope with misfolded proteins.
Proteins are the "tools" of our cells – they are essential to all vital tasks. However, they are only able to do their jobs if they fold correctly and adopt their respective, very specific 3D structure. To ensure that nothing goes wrong with the folding process, it is strictly monitored in the cell. The consequences of a flawed quality control can be seen, for example, in the deposition of misfolded proteins in neurodegenerative diseases such as Alzheimer's. Researchers at the Max Planck Institutes of Neurobiology and of Biochemistry have now developed a mouse line that makes the state of protein balance visible in the mammalian brain for the first time. In this way, the processes of protein quality control can now be studied in healthy and diseased neurons in more detail.
Proteins fulfill all important tasks in our body: They transport substances, protect against diseases, support the cell and catalyze chemical reactions – to name just a few. With the building instructions in our genetic code, every protein can be produced as a long chain of amino acids. However, that's not the end of the story: in order to perform their vital functions, proteins have to fold into complex 3D structures.
Each cell contains a whole machinery that helps proteins to fold, corrects folding errors and discards misfolded proteins. As a kind of quality control, the system thus contributes to proteostasis – the controlled function of all proteins.