Abstract 2700 Stability of Metal Binding Domains of Wilson Disease Protein

Wilson disease protein (WLNP) is a P1b-type ATPase crucial for maintaining copper homeostasis in humans.1 Mutations in this protein result in the autosomal recessive disorder Wilson disease. There are six metal-binding domains (MBDs) in WLNP, found within the first 650 amino acids of this 1,465 residue protein. The manner in which the six MBDs communicate with each other and how they affect other cytosolic-facing domains of WLNP is not understood. To better understand how the first four MBDs function, a detailed biophysical characterization of these domains was pursued.