Key Information
Source
American College of Cardiology Foundation
Year
2021
summary/abstract
Cardiac amyloidosis (CA) is a protein-folding disorder nearly exclusively caused by misfolded amyloid transthyretin (ATTR) and amyloid light chain (AL) proteins.
* Name derives from Latin "amylum" (starch like).
* Nomenclature: "A" for amyloid, followed by precursor protein abbreviation (e.g., AL = amyloid light chain amyloidosis).
* Histologic diagnosis: Aggregates of beta-sheets that stain with Congo red (green birefringence under polarized light). Congo red staining does not identify the type of amyloidosis.
* Systemic amyloidoses classified by precursor protein:
- Light chain and transthyretin (TTR) are the most common types.
- Immunoglobulin light chains are produced by the bone marrow plasma cell; circulating TTR (formerly known as "prealbumin") is produced by the liver.
- TTR can be genetically normal (transthyretin amyloidosis wild-type [ATTRwt]) or variant (transthyretin amyloidosis variant [ATTRv] or hereditary transthyretin amyloidosis [hATTR]).
- Single-nucleotide polymorphisms in the transthyretin (TTR) gene induce TTR instability and misfolding.
Abstract Source
https://www.acc.org/latest-in-cardiology/articles/2021/04/14/17/11/understanding-cardiac-amyloidosis?utm_medium=social&utm_source=twitter_post&utm_campaign=twitter_post