Wild-type (senile) amyloidosis pathophysiology: Difference between revisions
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*In systemic amyloidosis, [[amyloid]] gradually accumulates and [[amyloid]] deposition is widespread in the viscera, [[blood vessel]] walls, and different [[Connective tissue|connective tissues]].<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><ref name="pmid16409147">{{cite journal |vauthors=Pepys MB |title=Amyloidosis |journal=Annu. Rev. Med. |volume=57 |issue= |pages=223–41 |date=2006 |pmid=16409147 |doi=10.1146/annurev.med.57.121304.131243 |url=}}</ref> | *In systemic amyloidosis, [[amyloid]] gradually accumulates and [[amyloid]] deposition is widespread in the viscera, [[blood vessel]] walls, and different [[Connective tissue|connective tissues]].<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><ref name="pmid16409147">{{cite journal |vauthors=Pepys MB |title=Amyloidosis |journal=Annu. Rev. Med. |volume=57 |issue= |pages=223–41 |date=2006 |pmid=16409147 |doi=10.1146/annurev.med.57.121304.131243 |url=}}</ref> | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
* Wild-type (senile) amyloidosis is a type of systemic amyloidosis. | * Wild-type (senile) amyloidosis is a type of systemic amyloidosis as transthyretin (TTR) deposits can be found throughout the body. | ||
* The culprit protein responsible for the disease is | * The culprit protein responsible for the disease is TTR and it is deposited in the non-mutated form, hence the name "Wild-type". | ||
* The normal TTR protein compared with the mutated form is less likely to get deposited and | *TTR results in pathologies due to misfolding, breaking apart, and deposition of the amyloid fibrils in healthy tissue. | ||
* The normal TTR protein, compared with the mutated form, is less likely to get deposited and cause pathology. | |||
*This is believed to be the reason as to why this condition almost always affects the elderly (65 years of age or older). | |||
*The condition mainly affects the heart. However, other organ systems, such as the nervous and musculoskeletal systems, can also be involved. | |||
== Genetics == | |||
* There are no genes implicated in the causality of wild-type (senile) amyloidosis. | |||
== Associated Conditions == | |||
* Aging is very strongly associated with wild-type (senile) amyloidosis. | |||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, 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]
- These abnormal amyloids are derived from misfolding and aggregation of normally soluble proteins.
- Amyloid deposition can disrupt tissue structure of involved organ and consequently leads to organ failure.[2]
Systemic Amyloidosis
- In systemic amyloidosis, amyloid gradually accumulates and amyloid deposition is widespread in the viscera, blood vessel walls, and different connective tissues.[3][4]
Pathogenesis
- Wild-type (senile) amyloidosis is a type of systemic amyloidosis as transthyretin (TTR) deposits can be found throughout the body.
- The culprit protein responsible for the disease is TTR and it is deposited in the non-mutated form, hence the name "Wild-type".
- TTR results in pathologies due to misfolding, breaking apart, and deposition of the amyloid fibrils in healthy tissue.
- The normal TTR protein, compared with the mutated form, is less likely to get deposited and cause pathology.
- This is believed to be the reason as to why this condition almost always affects the elderly (65 years of age or older).
- The condition mainly affects the heart. However, other organ systems, such as the nervous and musculoskeletal systems, can also be involved.
Genetics
- There are no genes implicated in the causality of wild-type (senile) amyloidosis.
Associated Conditions
- Aging is very strongly associated with wild-type (senile) amyloidosis.
References
- ↑ Wechalekar AD, Gillmore JD, Hawkins PN (June 2016). "Systemic amyloidosis". Lancet. 387 (10038): 2641–2654. doi:10.1016/S0140-6736(15)01274-X. PMID 26719234.
- ↑ Wechalekar AD, Gillmore JD, Hawkins PN (June 2016). "Systemic amyloidosis". Lancet. 387 (10038): 2641–2654. doi:10.1016/S0140-6736(15)01274-X. PMID 26719234.
- ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ Pepys MB (2006). "Amyloidosis". Annu. Rev. Med. 57: 223–41. doi:10.1146/annurev.med.57.121304.131243. PMID 16409147.