X-RAY ABSORPTION STUDIES OF MUTANTS OF COPPER/ZINC SUPEROXIDE DISMUTASE

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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. X-ray absorption spectroscopy (XAS) will be used to investigate a series of copper-zinc superoxide dismutase (CuZnSOD) mutants. These mutants all exhibit a decrease in SOD activity and also show perturbations in the EPR and UV-vis spectra of the oxidized enzymes. Cu(II) K-edge XAS will allow for further characterization of the electronic and geometric structure of the oxidized enzyme. Cu(I) K-edge XAS will allow for characterization of the reduced enzyme, which will aid in understanding any alteration in the mechanism in these mutants. Zn K-edge XAS will be used to determine if any changes have occurred at the structural Zn site. These results will be compared to XAS studies on the WT enzyme and may provide important insight into mutant SOD1 enzyme toxicity in amyotrophic lateral sclerosis (ALS).