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 extracellular fibrillar protein that is insoluble and deposit in the tissues and causes disturbance of organ function and a wide variety of clinical syndromes that are classified according to the respective fibril protein precursor.[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 aligned in an antiparallel pattern. These sheets form rigid, nonbranching fibrils that resist proteolysis and eventually cause mechanical disruption and local oxidative stress in affected organs.
Systemic Amyloidosis
- In systemic amyloidosis, amyloid deposition is widespread and amyloid typically accumulate gradually.
Primary/Hereditary Amyloidosis
Secondary Amyloidosis
- Approximately 45% of generalized amyloidosis are secondary or reactive (AA) amyloidosis.[3]
- Pathogenesis of secondary amyloidosis is multifactorial involving many variables such as:
- Primary structure of the precursor protein
- Acute phase response
- Presence of non-fibril proteins (amyloid P component, apolipoprotein E, glycosaminoglycans, 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:[4]
- 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.
- ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ 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.
- ↑ 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.