title: Polymorphism of Amyloid Fibrils In Vivo.
abstract: Polymorphism is a wide-spread feature of amyloid-like fibrils formed in vitro, but it has so far remained unclear whether the fibrils formed within a patient are also affected by this phenomenon. In this study we show that the amyloid fibrils within a diseased individual can vary considerably in their three-dimensional architecture. We demonstrate this heterogeneity with amyloid fibrils deposited within different organs, formed from sequentially non-homologous polypeptide chains and affecting human or animals. Irrespective of amyloid type or source, we found in vivo fibrils to be polymorphic. These data imply that the chemical principles of fibril assembly that lead to such polymorphism are fundamentally conserved in vivo and in vitro.
authors.affiliation: Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany.
authors.forename: Karthikeyan
authors.initials: K
authors.lastname: Annamalai
authors.affiliation: CF Protemics, Leibniz Institute on Aging-Fritz Lipmann Institute (FLI), Beutenbergstraße 11, 07745, Jena, Germany.
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Gührs
authors.affiliation: Institute of Human Genetics, Im Neuenheimer Feld 366, 69120, Heidelberg, Germany.
authors.forename: Rolf
authors.initials: R
authors.lastname: Koehler
authors.affiliation: Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Schmidt
authors.affiliation: Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany.
authors.forename: Henri
authors.initials: H
authors.lastname: Michel
authors.affiliation: Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany.
authors.forename: Cornelia
authors.initials: C
authors.lastname: Loos
authors.affiliation: Department of Pathology, University of California, San Diego, 9500 Gilman Drive, MC 0162, La Jolla, CA, 92093-0612, USA.
authors.forename: Patricia M
authors.initials: PM
authors.lastname: Gaffney
authors.affiliation: Department of Pathology, University of California, San Diego, 9500 Gilman Drive, MC 0162, La Jolla, CA, 92093-0612, USA.
authors.forename: Christina J
authors.initials: CJ
authors.lastname: Sigurdson
authors.affiliation: Amyloidosis Center, Department of Internal Medicine V, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany.
authors.forename: Ute
authors.initials: U
authors.lastname: Hegenbart
authors.affiliation: Amyloidosis Center, Department of Internal Medicine V, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Schönland
authors.affiliation: Institute of Protein Biochemistry, Ulm University, Helmholtzstrasse 8/1, 89081, Ulm, Germany. marcus.faendrich@uni-ulm.de.
authors.forename: Marcus
authors.initials: M
authors.lastname: Fändrich
mesh_terms: D000682:Amyloid / Q000737:chemistry*; D046529:Microscopy, Electron, Transmission; D011487:Protein Conformation
chemical_list: D000682:Amyloid
keywords: Alzheimer's disease; Parkinson's disease; prions; protein folding; systemic amyloidosis