History
The syndrome was first described in 1936 by the Austrian neurologists Josef Gerstmann, Ernst Sträussler, and I. Scheinker. The syndrome is now known to be a form of transmissible spongiform encephalopathy (TSE) -- a prion disease.
Overview
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Proteins are functionally important molecules in cells. They are made of amino acids, "strung together" with peptide bonds, and folded into a 3-D structure according to the chemical bonding properties of the amino acids. They are flexible molecules, which can change conformation (shape) depending on the environment they are in or the function they are performing; however, they normally keep one general shape. Proteins have different levels of structure. The primary structure is the amino acid sequence. The secondary structures are different shapes that regions of proteins may take-for example, alpha helices or beta sheets. Alpha helices are right-handed spirals and beta sheets are made up of multiple parallel strands that look like pleated sheets. The tertiary structure of a protein involves the rest of the folding of the protein into a complete three-dimensional structure, and the quaternary structure is the way in which proteins may interact, for example proteins coming together to form a larger unit.
GSS History
GSS is a disease that is very rare, making its history hard to track exactly where it descended from. Gerstmann-Straussler-Scheinker syndrome (GSS) were first described as neurodegenerative diseases in the 1920s. Gerstmann-Straussler-Scheinker (GSS) is called this because it was first reported in 1936 by Josef Gerstmann, Ernst Straussler and Ilya Scheinker. They were all German doctors who were specialized in prion protein diseases. In 1989, the first mutation of the prion protein gene was identified in a GSS family. Prion diseases are rare degenerative diseases of the brain thought to be caused by a protein that converts to an abnormal form called a prion. GSS was later realized to have many different gene mutation types, with some showing different symptoms first or having other symptoms worse than others. Doctors in different parts of the world are recovering more generations and families that have the mutation. It is hard to discover GSS for two main reasons: the disease has been reported in only a few countries; and, the disease may be underreported due to its clinical similarity to other diseases. The Indiana Kindred is the largest, spanning over 8 generations, and includes over 3,000 people with 57 individuals known to be affected.