The consortium has different mouse models for HSP available for the planned metabolic investigations. These animals will be bred in sufficient numbers and killed at defined times in order to remove brain and spinal cord tissue as well as cerebrospinal fluid and blood. In these samples, a commercial provider will quantify all characteristic metabolic properties and searches for changes in diseased animals. These data will then be used to identify metabolic pathways that are disturbed in the disease and to further validate them through appropriate functional and biochemical investigations. In the next step, this will allow the identification of biomarkers, i.e. measurable substances that correlate as closely as possible with the disease stage. Such markers will then be investigated in different tissues and body fluids of healthy and diseased animals over time in order to clarify whether these markers can possibly be used in early stages of disease to differentiate between diseased and wildtype animals.