Key Information
The Nomenclature Committee of the International Society of Amyloidosisexternal link, opens in a new tab 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.
Three are polypeptide hormones, and two are currently used peptide drugs, bringing the total number of known iatrogenic amyloid forms to four, all of which manifest as subcutaneous nodules at the injection site. The sixth novel amyloid fibril protein is the transmembrane 106B protein, which forms intracellular amyloid fibrils in disorders associated with frontotemporal dementia. The number of known human amyloid fibril proteins is now 42.