Noncerebral Amyloidoses: Aspects on Seeding, Cross-Seeding, and Transmission

  1. Per Westermark4
  1. 1Department of Medical Cell Biology, Uppsala University, SE-751 23 Uppsala, Sweden
  2. 2Institute of Protein Biochemistry, Ulm University, D-89081 Ulm, Germany
  3. 3Department of Clinical Pathology and Clinical Genetics, and Department of Clinical and Experimental Medicine, Linköping University, SE-581 85 Linköping, Sweden
  4. 4Department of Immunology, Genetics and Pathology, Uppsala University, SE-751 85 Uppsala, Sweden
  1. Correspondence: per.westermark{at}igp.uu.se

Abstract

More than 30 proteins form amyloid in humans, most of them outside of the brain. Deposition of amyloid in extracerebral tissues is very common and seems inevitable for an aging person. Most deposits are localized, small, and probably without consequence, but in some instances, they are associated with diseases such as type 2 diabetes. Other extracerebral amyloidoses are systemic, with life-threatening effects on the heart, kidneys, and other organs. Here, we review how amyloid may spread through seeding and whether transmission of amyloid diseases may occur between humans. We also discuss whether cross-seeding is important in the development of amyloidosis, focusing specifically on the amyloid proteins AA, transthyretin, and islet amyloid polypeptide (IAPP).

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