Several human neurodegenerative diseases, including Alzheimer’s, Parkinson’s and Huntington’s disease but also ageing, are linked to an accumulation of abnormal and aggregated proteins in cells. Cellular “garbage” of this type can be removed from cells by sweeping them to a cellular recycling station known as the lysosome.
Scientists at the Max Planck Institute of Biochemistry in Martinsried, Germany, now discovered a new family of helper proteins that recognize labeled cellular protein waste and guide them efficiently to the lysosome for destruction and subsequent recycling into their reusable compounds.
The results of this study, now published in the journal Cell, are crucial for our understanding how cells remove cellular waste and will open new avenues for studies aimed to fight neurodegenerative diseases.
Proteins, the components of our body that execute, control and organize basically all functions in our cells, are made out of strings of amino acids, which – like an origami – are folded into specific and complex three-dimensional structures according to their desired functions. However, since folding and maintaining of such structures is highly sensitive to cellular or environmental stress, proteins can potentially misfold or form clumps (aggregates). Such undesired protein waste can be toxic for cells and may even lead to cell death. Because several human neurodegenerative diseases are known to be linked to an accumulation of abnormal protein aggregates, basic science aimed to understand how cells remove cellular garbage is elementary for designing strategies for a potential prevention or cure of such disorders.