PRIMARY AMYLOIDOSIS: CON FORM ATiONAL INTERMEDIATES IN IMMUNOGLOBULIN LIGHT CHAIN PROTEINS UPON INTERACTION WITH CONGO RED UNDER PHYSIOLOGICAL CONDITIONS

摘要

Congo red (CR) is a dye used to identify amyloid fibril deposits in tissues (1). CR and its structural analogs have also been suggested as potential therapeutic agents (2). CR has been shown to block amyloid fibril formation and reverse fibril deposition in an animal model of Huntington's disease by unknown mechanisms (3). CR binding to Immunoglobulin Light Chains (IgLCs) may lead to partial unfolding and conformational intermediates. Our goal was to identify and biophysically characterize conformationa! intermediates in soluble IgLCs upon interaction with CR.