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Flashcards in Amylodosis Deck (27):
1

amyloid

pathological proteinaceous substance deposited in extracellular space in various tissues and organs of the body

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what is an amyloid

amorphous
eosinophils
hyaline

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how does amyloid damage?

with progressive accumulation it encroaches on and produces pressure atrophy of adjacent cells. it itself is not toxic

4

amyloidosis

should not be considered a signle disease--group of diseases having in common deposition of similar appearing proteins

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amyloid protein is made up of

continuous, nonbranching fibrils in a cross-B-pleated sheet conformation (responsible for distinctive congo red staining) -95%

remaining 5%- P component and other glycoproteins

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5 types of amyloidosis

primary amy
secondary amy
familial amy
senile systemic amy
dialysis amy

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primary amyloidosis

K or gamma light chain/AL type
--multiple myeloma
but most cases do not have neoplasms

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secondary amyloidosis

protein A (major component of amyloid fibril)
--derived frm acute phase protein during chronic inflammation
--serum amyloid A (precursor) is elevated with rheumatoid arthritis and Crohn's disease

9

3 types of familial amyloidosis

neuropathic
cardiopathic
nephropathic

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neuropathic familial amylodosis

portgual, sweden, japan, and other countries
-transthyretin mutant (prealbumin)
-->peripheral neuropathy; sub of methionine for valine at residue 30

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cardiomyopathy familial amylodosis

denmark and appalachia in US
-transthyretin mutant (prealbumin)

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renal familial amylodosis

mediterranean fever
issue with neutrophils--> acute infammation because of this malfunction

mutation of fibrinogen a chain (leu 554 or Glu 526), or mutations of fibronogen a chain (protein a)
"autoinflammatory"-high IL1

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senile systemic amylodosis

senile cardiac
transthryetin (TTR) normal (prealbumin)
ASYMPTOMATIC

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dialysis amyloidosis

B2 microglobulin deposition

15

unknown carcinogen stimulus

monoclonal b lymphocyte proliferation-->plasma cells-
sol precursor misfolded protein: immunoglobulin light chain--> limited proteolysis-->AL protein

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chronic inflammation stimulus

MO activation-->IL1 and IL6-->liver cells

sol. misfolded precursor preotein: Saa protein-->limited proteolysis-->AA protein

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lichen amyloidosis

nodular amyloid deposits in skin

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congo red makes amyloid appear

granny apple smith green

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ATTR protein

what is made from normal ttr
mutation-->mutant ttr-->aggregation-->ATTR rotein

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proteins of amyloid

AL-amyloid light chain
AA-amyloid-associated
AB amyloid
Mutant Transthyretin (TTR)
B2 microglobulin
Prions

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AA protein derived from

unique non-ig protein synthesized in liver

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AB amyloid

produced from B amyloid precursor protein found in cerebral lesions of alzheimer disease

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amyloid proteins fall in two categories

normal proteins that have inherent tendency to fold improperly, associate and form fibrils

mutant proteins that are prone to misfolding and subsequent aggregation

24

primary vs secondary amyloidosis

primary: associated with immunocyte disorder
secondary: complication of an underlying chronic inflammatory or tissue-destructive process

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what is primary amyloidosis associated with?

plasma cell dyscrasia

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most common organ of deposition

kidney- nephrotic syndrome (proteinuria)

27

type II diabetes

deposition of amylin in islets of pancreas