title: Amyloid persistence in decellularized liver: biochemical and histopathological characterization.
abstract: Systemic amyloidoses are a group of debilitating and often fatal diseases in which fibrillar protein aggregates are deposited in the extracellular spaces of a range of tissues. The molecular basis of amyloid formation and tissue localization is still unclear. Although it is likely that the extracellular matrix (ECM) plays an important role in amyloid deposition, this interaction is largely unexplored, mostly because current analytical approaches may alter the delicate and complicated three-dimensional architecture of both ECM and amyloid. We describe here a decellularization procedure for the amyloidotic mouse liver which allows high-resolution visualization of the interactions between amyloid and the constitutive fibers of the extracellular matrix. The primary structure of the fibrillar proteins remains intact and the amyloid fibrils retain their amyloid enhancing factor activity.
authors.affiliation: a Institute for Liver and Digestive Health .
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Mazza
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.
authors.forename: J Paul
authors.initials: JP
authors.lastname: Simons
authors.affiliation: c Centre for Biomedical Science, Division of Medicine, University College London , London , UK .
authors.forename: Raya
authors.initials: R
authors.lastname: Al-Shawi
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.
authors.forename: Stephan
authors.initials: S
authors.lastname: Ellmerich
authors.affiliation: d Stem Cells and Regenerative Medicine Section, Developmental Biology and Cancer Programme, UCL Institute for Child Health, Great Ormond Street Hospital, University College London , London UK .
authors.forename: Luca
authors.initials: L
authors.lastname: Urbani
authors.affiliation: e Department of Molecular Medicine , Institute of Biochemistry, University of Pavia , Pavia , Italy , and.
authors.forename: Sofia
authors.initials: S
authors.lastname: Giorgetti
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.
authors.forename: Graham W
authors.initials: GW
authors.lastname: Taylor
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.
authors.forename: Janet A
authors.initials: JA
authors.lastname: Gilbertson
authors.affiliation: a Institute for Liver and Digestive Health .
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Hall
authors.affiliation: a Institute for Liver and Digestive Health .
authors.forename: Walid
authors.initials: W
authors.lastname: Al-Akkad
authors.affiliation: a Institute for Liver and Digestive Health .|f Organ Transplantation Centre and Comparative Medicine Department, King Faisal Specialist Hospital , Riyadh , Saudi Arabia.
authors.forename: Dipok
authors.initials: D
authors.lastname: Dhar
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: d Stem Cells and Regenerative Medicine Section, Developmental Biology and Cancer Programme, UCL Institute for Child Health, Great Ormond Street Hospital, University College London , London UK .
authors.forename: Paolo
authors.initials: P
authors.lastname: De Coppi
authors.affiliation: a Institute for Liver and Digestive Health .
authors.forename: Massimo
authors.initials: M
authors.lastname: Pinzani
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.|e Department of Molecular Medicine , Institute of Biochemistry, University of Pavia , Pavia , Italy , and.
authors.forename: Vittorio
authors.initials: V
authors.lastname: Bellotti
authors.affiliation: b Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins , and.|e Department of Molecular Medicine , Institute of Biochemistry, University of Pavia , Pavia , Italy , and.
authors.forename: P Patrizia
authors.initials: PP
authors.lastname: Mangione
mesh_terms: D000595:Amino Acid Sequence; D000682:Amyloid / Q000737:chemistry / Q000502:physiology*; D000686:Amyloidosis / Q000473:pathology*; D000818:Animals; D005109:Extracellular Matrix / Q000502:physiology; D005260:Female; D000075363:Immunoglobulin Light-chain Amyloidosis; D008099:Liver / Q000737:chemistry / Q000473:pathology*; D008107:Liver Diseases / Q000473:pathology*; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D008969:Molecular Sequence Data; D000685:Serum Amyloid A Protein / Q000737:chemistry
chemical_list: D000682:Amyloid; D000685:Serum Amyloid A Protein
keywords: AA amyloidosis; AEF; amyloid; decellularized liver; scaffold