Amyloidogenic potential of foie gras.
The human cerebral and systemic amyloidoses and prion-associated spongiform encephalopathies are acquired or inherited protein folding disorders in which normally soluble proteins or peptides are converted into fibrillar aggregates. This is a nucleation-dependent process that can be initiated or accelerated by fibril seeds formed from homologous or heterologous amyloidogenic precursors that serve as an amyloid enhancing factor (AEF) and has pathogenic significance in that disease may be transmitted by oral ingestion or parenteral administration of these conformationally altered components. Except for infected brain tissue, specific dietary sources of AEF have not been identified. Here we report that commercially available duck- or goose-derived foie gras contains birefringent congophilic fibrillar material composed of serum amyloid A-related protein that acted as a potent AEF in a transgenic murine model of secondary (amyloid A protein) amyloidosis. When such mice were injected with or fed amyloid extracted from foie gras, the animals developed extensive systemic pathological deposits. These experimental data provide evidence that an amyloid-containing food product hastened the development of amyloid protein A amyloidosis in a susceptible population. On this basis, we posit that this and perhaps other forms of amyloidosis may be transmissible, akin to the infectious nature of prion-related illnesses.
Solomon
Alan
A
Human Immunology and Cancer Program, Department of Medicine, University of Tennessee Graduate School of Medicine, Knoxville, TN 37920, USA. asolomon@mc.utmck.edu
Richey
Tina
T
Murphy
Charles L
CL
Weiss
Deborah T
DT
Wall
Jonathan S
JS
Westermark
Gunilla T
GT
Westermark
Per
P
D000686:Amyloidosis / Q000139:chemically induced / Q000209:etiology*; D000818:Animals; D004195:Disease Models, Animal; D004372:Ducks; D005777:Geese; D006023:Glycoproteins / Q000008:administration & dosage / Q000494:pharmacology; D008461:Meat Products / Q000009:adverse effects*; D051379:Mice; D008822:Mice, Transgenic; D000685:Serum Amyloid A Protein / Q000008:administration & dosage* / Q000494:pharmacology
D006023:Glycoproteins; D000685:Serum Amyloid A Protein; C046748:amyloid enhancing factor