AI Article Synopsis
- Amyloidosis is a disorder where misfolded proteins accumulate in tissues, leading to organ failure and potentially death, affecting places like the heart, liver, and kidneys.
- Over 60 amyloid proteins exist, with around 30 linked to human diseases, characterized by their formation of rigid beta-pleated sheets that resist breakdown.
- The review focuses on light chain amyloidosis, covering its prevalence, staging, and prognosis to guide effective treatment options.
Article Abstract
Amyloidosis is a disorder of protein misfolding and metabolism in which insoluble fibrils are deposited in various tissues, causing organ dysfunction and eventually death. Out of the 60-plus heterogeneous amyloidogenic proteins that have been identified, approximately 30 are associated with human disease. The unifying feature of these proteins is their tendency to form beta-pleated sheets aligned in an antiparallel fashion. These sheets then form rigid, nonbranching fibrils that resist proteolysis, causing mechanical disruption and local oxidative stress in affected organs such as the heart, liver, kidneys, nervous system, and gastrointestinal tract. Here we review the epidemiology of light chain amyloidosis, the staging, and the concomitant prognostication that is critical in determining the appropriate treatment.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8932379 | PMC |
| http://dx.doi.org/10.14797/mdcvj.1070 | DOI Listing |
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