NON-INVASIVE QUANTITATION OF CELLU & BIOCHEM CHANGES IN SPINOCEREBELLAR ATAXIAS

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. (in collaboration with Dr.s Khalaf Bushara, Neurology and Christopher Gomez, University of Chicago, Neurology, supported by R21 NS056172, philanthropic funds and Bob Allison Ataxia Research Center, PI Oz): In this project we study the neurochemical alterations that are associated with neurodegeneration in spinocerebellar ataxias (SCAs). The goal is to establish and validate markers of multiple cellular and biochemical events associated with neurodegeneration, such as neuronal death, gliosis and oxidative stress. We have so far scanned patients with SCA1, SCA2, SCA6, Friedreich's ataxia, cerebellar multiple system atrophy and ataxia with oculomotor apraxia type 2, as well as age-matched healthy controls. We have detected alterations in the neurochemical profile in ataxias by high field nuclear magnetic resonance spectroscopy (MRS) and found that these are specific for different ataxia subtypes.