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14 Cards in this Set
- Front
- Back
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What are the Amyloidoses?
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A group of diseases with extracellular accumulation of fibrillar protein deposits.
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What causes the extracellular protein aggregates to form in amyloidosis?
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Misfolding
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Are the misfolded proteins mutated in amyloidosis?
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Not necessarily; they can be.
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What should happen to proteins normally when they're misfolded?
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Proteolysis
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Why aren't the misfolded proteins that aggregate and cause amyloidosis proteolysed?
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Because they're either resistant to proteolysis, or the proteolytic enzyme may not achieve complete proteolysis.
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What is the diagnostic test for amyloidosis?
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Apple green birefringence of Congo red dye under polarized light.
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What is Amyloid AA associated with?
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Inflammation
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What is Amyloid AL associated with? What is the protein precursor that accumulates?
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Plasma cell dyscrasia
-Kappa or lambda light chains |
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What is Amyloid A-beta?
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-Alzheimer's
-Congophilic angiopathy |
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What is Amyloid-Abeta2M?
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Chronic dialysis induced aggregation of B2microglobulin
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What do 89% of patients with AL amyloidosis have? What do 70% have?
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Mprotein = 89%
Lambda = 70% |
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What do 5-15% of patients with myeloma have?
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Coexistent amyloidosis
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What are 5 complications of amyloid deposition?
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-Nephrotic syndrome or renal insufficiency
-Congestive heart failure -Conduction disturbances and arrythmias -Peripheral neuropathy -Carpal tunnel syndrome |
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What are 4 other complications that may be seen in amyloidosis?
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-Hepatomegaly
-Splenomegaly -Enlargment of the tongue -Idiopathic malabsorption diarrhea or digestion problems |
