Amyloid Flashcards
What is amyloid?
a class of insoluble abnormal protein deposits in tissue
What is amyloidosis?
disease condition where amyloid is deposited locally or systemically
Name the 2 classifications of amyloidosis
- systemic - involves more than one organ
2. localized - restricted to a single organ or tissue
How is amyloid identified grossly?
iodine-sulfuric acid method to stain amyloid deposits in tissue at necropsy
How is amyloid identified microscopically?
H&E stain
Congo red stain
Thioflavin S or T
What is the structure/composition of amyloid?
- fibrils
- protein secondary structure beta-pleated sheets
What is the significance of replacement of functional tissue with amyloid?
- physical compression of normal tissues resulting in atrophy
- disruption of normal structures and impaired function
Nomenclature of amyloid
“A” (for amyloid) followed by abbreviation for protein precursor (ex: AA, AL, etc.)
What is the significance of cytotoxic amyloid oligomers and/or fibrils?
- induce cell stress responses and apoptosis
Pathogenesis of amyloidosis
- increased production of precursor protein
- mutation in precursor protein resulting in more amyloidogenic form
- abnormal processing of precursor protein to form amyloidogenic polypeptides
Characteristics of AA (secondary/reactive) amyloidosis
- most serious systemic form in mammals and birds
- occurs secondary to recurrent or chronic disease
- increased hepatic synthesis of SAA
- macrophage degradation of SAA to amyloidogenic fragment
Clinical signs of systemic AA amyloidosis
- renal glomerulus problems
- marked proteinuria and renal failure
- hepatic failure may develop with severe liver involvement
Which gene mutation has recently been linked to Shar Pei AA amyloidosis?
HAS2 gene
What is the second form of canine amyloidosis?
- pulmonary vascular amyloid (most common systemic form)
- found in large proportion of aged dogs
- derived from apolipoprotein A1
what is the third form of canine amyloidosis?
- beta-protein derived (Alzheimer’s-like) cerebral amyloid
- associated with cognitive dysfunction