Amyloid nomenclature 2022: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee

TLDR

The nomenclature principles remain unchanged, though debate continues about the significance and diversity of intracellular protein aggregates, especially in neurodegenerative diseases. The ISA Nomenclature Committee met in September and October 2022, producing this upgraded nomenclature recommendation. Six new amyloid fibril proteins were added—three polypeptide hormones, two peptide drugs (raising iatrogenic forms to four, all presenting as subcutaneous nodules), and the transmembrane 106B protein linked to frontotemporal dementia—bringing the total to 42.

Abstract

The Nomenclature Committee of the International Society of Amyloidosis met at the XVIII International Symposium on Amyloidosis in September and virtually in October 2022 with discussions resulting in this upgraded nomenclature recommendation. The nomenclature principles remain unchanged but there is an ongoing discussion regarding the importance and varying nature of intracellular protein aggregates, particularly those associated with neurodegenerative diseases. Six novel proteins were added to the list of human amyloid fibril proteins. Of these, three are polypeptide hormones and two currently utilised peptide drugs, making the number of known iatrogenic amyloid forms four, all appearing as subcutaneous nodules at the injection site. The sixth novel amyloid fibril protein is the transmembrane 106B protein, forming intracellular amyloid fibrils in disorders associated with frontotemporal dementia. The number of known human amyloid fibril proteins is now 42.