Amyloidosis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
Overview
Pathophysiology
- Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes.[1][2]
- Approximately 27 amyloidogenic proteins that are associated with known human disease have been identified. The mutual feature of these proteins is their tendency to form β-pleated sheets that arranged in an antiparallel pattern. These sheets form rigid, non-branching fibrils that are proteolysis resistant. These sheets eventually cause mechanical disruption and local oxidative stress in affected organs.
Systemic Amyloidosis
Primary/Hereditary Amyloidosis
Secondary Amyloidosis
- Secondary or reactive amyloidosis (AA) is approximately 45% of all systemic amyloidosis.[3]
- Pathogenesis of secondary or reactive amyloidosis is multifactorial that include:
- Primary structure of the precursor protein
- Acute phase response
- Nonfibril proteins (amyloid P component, apo E, GAGs, proteoglycans and basement membrane proteins)
- Receptors
- Lipid metabolism
- Proteases
Organ-specific Amyloidosis
Gross Pathology
Microscopic Pathology
In microscopy pathology of amyloidosis, amyloid is detectable as:[3]
- Typical green birefringence in polarised light after staining with Congo red
- Non-branching linear fibrils (indefinite length with an approximate diameter of 10-12 nm)
- Distinct X-ray diffraction pattern consistent with Pauling's model of a cross-beta fibril
Other Diseases Associated with the Amyloid Protein
References
- ↑ Gillmore JD, Hawkins PN (October 2013). "Pathophysiology and treatment of systemic amyloidosis". Nat Rev Nephrol. 9 (10): 574–86. doi:10.1038/nrneph.2013.171. PMID 23979488.
- ↑ 2.0 2.1 Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ 3.0 3.1 Röcken C, Shakespeare A (February 2002). "Pathology, diagnosis and pathogenesis of AA amyloidosis". Virchows Arch. 440 (2): 111–122. doi:10.1007/s00428-001-0582-9. PMID 11964039.