Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

Merrill D. Benson, Joel N. Buxbaum, David S. Eisenberg, Giampaolo Merlini, Maria J.M. Saraiva, Yoshiki Sekijima, Jean D. Sipe, Per Westermark

Research output: Contribution to journalArticlepeer-review

Abstract

The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September 2020 due to the ongoing COVID-19 pandemic instead of a planned meeting in Tarragona in March. In addition to confirmation of basic nomenclature, several additional concepts were discussed, which are used in scientific amyloid literature. Among such concepts are cytotoxic oligomers, protofibrils, primary and secondary nucleation, seeding and cross-seeding, amyloid signature proteins, and amyloid plaques. Recommendations for their use are given. Definitions of amyloid and amyloidosis are confirmed. Possible novel human amyloid fibril proteins, appearing as ‘classical’ in vivo amyloid, were discussed. It was decided to include fibulin-like extracellular matrix protein 1 (amyloid protein: AEFEMP1), which appears as localised amyloid in portal veins. There are several possible amyloid proteins under investigation, and these are included in a new Table.

Original languageEnglish (US)
Pages (from-to)217-222
Number of pages6
JournalAmyloid
Volume27
Issue number4
DOIs
StateAccepted/In press - 2020

Keywords

  • Amyloid
  • aggegation
  • fibril protein
  • inclusion
  • nomenclature
  • oligomer

ASJC Scopus subject areas

  • Internal Medicine

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