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The discovery of prion disease and the establishment of the protein only hypothesis of prion propagation raised substantial interest in the class of maladies referred to as conformational diseases. Although significant pro-gress has been made in elucidating the mechanisms of polymerization for several amyloidogenic proteins and peptides linked to conformational dis- | MINIREVIEW Branched chain mechanism of polymerization and ultrastructure of prion protein amyloid fibrils Ilia V. Baskakov1 2 1 MedicalBiotechnology Center University of Maryland Biotechnology Institute Baltimore MD USA 2 Department of Biochemistry and Molecular Biology University of Maryland Schoolof Medicine Baltimore MD USA Keywords amyloid fibrils branched-chain mechanism in vitro conversion polymerization kinetics prion diseases prion protein Correspondence I. V. Baskakov 725 West Lombard Street Baltimore MD 21201 USA Fax 1 410 706 8184 Tel 1 410 706 4562 E-mail baskakov@umbi.umd.edu Received 9 March 2007 accepted 31 May 2007 The discovery of prion disease and the establishment of the protein only hypothesis of prion propagation raised substantial interest in the class of maladies referred to as conformational diseases. Although significant progress has been made in elucidating the mechanisms of polymerization for several amyloidogenic proteins and peptides linked to conformational disorders and solving their fibrillar 3D structures studies of prion protein amyloid fibrils and their polymerization mechanism have proven to be very difficult. The present minireview introduces the mechanism of branched-chain reaction for describing the peculiar kinetics of prion polymerization and summarizes our current knowledge about the substructure of prion protein amyloid fibrils. doi 10.1111 j.1742-4658.2007.05916.x Prion diseases are a group of fatal neurodegenerative maladies that can arise spontaneously or be inherited and that can also be infectious 1 . Despite enormous investments over the last 30 years in searching for a prion virus or virion 2-5 no prion-specific nucleic acids associated with infectious prion particles have ever been identified 6 . A notable shift has occurred in the last few years from debating the question of whether a protein can be infectious to what makes a protein infectious and how many proteins are infectious 7-9 . Elucidating the .
