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SYNCHROTRON--SUPEROXIDE DISMUTASE, DIPHTHERIA TOXIN, DE NOVO DESIGNED PEPTIDES

$143,176P41FY2002RRNIH

Stanford University, Stanford CA

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Abstract

Amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) is neurodegenerative disorder characterized by the progressive destruction of large motor neurons in the spinal cord and brain. Approximately 10% of ALS-afflicted individuals having the inherited (familial) form of the disease (FALS) display tight genetic linkage between the disorder and a gene encoding a cytosolic Cu/Zn-binding superoxide dismutase (SOD1). We desire to correlate ALS misfunction withCu/ZnSOD structure via crystallographic analysis of human SOD mutants found in FALS patients.

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