US scientist researched molecular bases of Alzheimer and BSE

When cows are stricken with BSE or people with Parkinson’s  or Alzheimer’s disease, the causes – as far as we know today – are in principle very similar: they take the form of “malformations” on the molecular level. Certain protein molecules have folded incorrectly and adopted a pathogenic conformation. The US scientist Susan Lindquist has made seminal contributions towards understanding the mechanisms of protein misfolding underlying these disease states.. In recognition of her work,  Lindquist was awarded the Otto Warburg Medal, Germany’s most prestigious award for biochemists and molecular biologists. The prize ceremony took place on March 27th, 2008 in Mosbach, a small town in Baden-Württemberg, Germany.

Proteins are generally considered the “building blocks of life”. Unlike building blocks though they are not rigid, but flexible and can take on a whole range of completely different three-dimensional structures. The exact 3D arrangement of the chain-like molecules, or their so-called folding, is of crucial importance. Importantly, these structural arrangements are very delicate and can be disturbed by a variety of stresses such as heat or toxic substances.

Professor Susan Lindquist of the Massachusetts Institute of Technology (MIT) in Cambridge, USA has been fascinated by how cells defend themselves against such stress phenomena and manage to maintain their proteins in a properly folded and biologically active state. She found that the cellular stress response,  the defence mechanism against misfolded proteins, is critical in deciding whether a cell survives under conditions that lead to increased production of incorrect proteins.  This is most relevant in the so-called protein folding diseases, includingAlzheimer’s and Parkisnon’s disease, and also the prion diseases, a kind of infectious protein misfolding process that causes mad cow disease (BSE) and Creutzfeld-Jakob disease. In all these, presently incurable, neurodegenerative diseases, certain proteins lose their proper shape and clump together as useless aggregates that eventually kill the affected cells.

“Susan Lindquist has made groundbreaking discoveries in understanding the cellular stress response against misfolded proteins,” explained Professor Alfred Wittinghofer, President of the German Biochemistry and Molecular Biology Society (GBM). “Many human and animal diseases can only be understood in light of  her findings. And if there may perhaps be a cure for these diseases one day, it will build on her crucial contributions.”