Amyloidosis pathophysiology: Difference between revisions
Jump to navigation
Jump to search
| Line 7: | Line 7: | ||
==Pathophysiology== | ==Pathophysiology== | ||
* Amyloid is an abnormal extracellular | * Amyloid is an abnormal insoluble extracellular protein that deposits in the different tissues and causes organic dysfunction and a wide variety of clinical syndromes.<ref name="pmid23979488">{{cite journal |vauthors=Gillmore JD, Hawkins PN |title=Pathophysiology and treatment of systemic amyloidosis |journal=Nat Rev Nephrol |volume=9 |issue=10 |pages=574–86 |date=October 2013 |pmid=23979488 |doi=10.1038/nrneph.2013.171 |url=}}</ref><ref name="pmid23227278">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref> | ||
* 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 | * 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=== | ===Systemic Amyloidosis=== | ||
* In systemic amyloidosis, amyloid deposition is widespread | * In systemic amyloidosis, [[amyloid]] gradually accumulate and amyloid deposition is widespread.<ref name="pmid23227278">{{cite journal |vauthors=Baker KR, Rice L |title=The amyloidoses: clinical features, diagnosis and treatment |journal=Methodist Debakey Cardiovasc J |volume=8 |issue=3 |pages=3–7 |date=2012 |pmid=23227278 |pmc=3487569 |doi= |url=}}</ref> | ||
====Primary/Hereditary Amyloidosis==== | ====Primary/Hereditary Amyloidosis==== | ||
==== Secondary Amyloidosis ==== | ==== Secondary Amyloidosis ==== | ||
* | * Secondary or reactive amyloidosis (AA) is approximately 45% of all systemic amyloidosis.<ref name="pmid119640392">{{cite journal |vauthors=Röcken C, Shakespeare A |title=Pathology, diagnosis and pathogenesis of AA amyloidosis |journal=Virchows Arch. |volume=440 |issue=2 |pages=111–122 |date=February 2002 |pmid=11964039 |doi=10.1007/s00428-001-0582-9 |url=}}</ref> | ||
* Pathogenesis of secondary amyloidosis is multifactorial | * [[Pathogenesis]] of secondary or reactive amyloidosis is multifactorial that include many variables such as: | ||
** Primary structure of the precursor protein | ** Primary structure of the precursor protein | ||
** Acute phase response | ** Acute phase response | ||
** | ** Nonfibril [[Protein|proteins]] (amyloid P component, [[Apolipoprotein E|apo E]], [[Glycosaminoglycan|glycosaminoglycans]], [[Proteoglycan|proteoglycans]] and [[basement membrane]] [[Protein|proteins]]) | ||
** Receptors | ** [[Receptor (biochemistry)|Receptors]] | ||
** Lipid metabolism | ** [[Lipid metabolism]] | ||
** Proteases | ** [[Protease|Proteases]] | ||
=== Organ-specific Amyloidosis === | === Organ-specific Amyloidosis === | ||
Revision as of 19:27, 7 May 2018
|
Amyloidosis Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Amyloidosis pathophysiology On the Web |
|
American Roentgen Ray Society Images of Amyloidosis pathophysiology |
|
Risk calculators and risk factors for Amyloidosis pathophysiology |
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 many variables such as:
- Primary structure of the precursor protein
- Acute phase response
- Nonfibril proteins (amyloid P component, apo 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:[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.