Amylodosis

Question 1

amyloid

Answer 1

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

Question 2

what is an amyloid

Answer 2

amorphous
eosinophils
hyaline

Question 3

how does amyloid damage?

Answer 3

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

Question 4

amyloidosis

Answer 4

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

Question 5

amyloid protein is made up of

Answer 5

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

remaining 5%- P component and other glycoproteins

Question 6

5 types of amyloidosis

Answer 6

primary amy
secondary amy
familial amy
senile systemic amy
dialysis amy

Question 7

primary amyloidosis

Answer 7

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

Question 8

secondary amyloidosis

Answer 8

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

Question 9

3 types of familial amyloidosis

Answer 9

neuropathic
cardiopathic
nephropathic

Question 10

neuropathic familial amylodosis

Answer 10

portgual, sweden, japan, and other countries

• transthyretin mutant (prealbumin)
• ->peripheral neuropathy; sub of methionine for valine at residue 30

Question 11

cardiomyopathy familial amylodosis

Answer 11

denmark and appalachia in US

-transthyretin mutant (prealbumin)

Question 12

renal familial amylodosis

Answer 12

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

Question 13

senile systemic amylodosis

Answer 13

senile cardiac
transthryetin (TTR) normal (prealbumin)
ASYMPTOMATIC

Question 14

dialysis amyloidosis

Answer 14

B2 microglobulin deposition

Question 15

unknown carcinogen stimulus

Answer 15

monoclonal b lymphocyte proliferation–>plasma cells-

sol precursor misfolded protein: immunoglobulin light chain–> limited proteolysis–>AL protein

Question 16

chronic inflammation stimulus

Answer 16

MO activation–>IL1 and IL6–>liver cells

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

Question 17

lichen amyloidosis

Answer 17

nodular amyloid deposits in skin

Question 18

congo red makes amyloid appear

Answer 18

granny apple smith green

Question 19

ATTR protein

Answer 19

what is made from normal ttr

mutation–>mutant ttr–>aggregation–>ATTR rotein

Question 20

proteins of amyloid

Answer 20

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

Question 21

AA protein derived from

Answer 21

unique non-ig protein synthesized in liver

Question 22

AB amyloid

Answer 22

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

Question 23

amyloid proteins fall in two categories

Answer 23

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

mutant proteins that are prone to misfolding and subsequent aggregation

Question 24

primary vs secondary amyloidosis

Answer 24

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

Question 25

what is primary amyloidosis associated with?

Answer 25

plasma cell dyscrasia

Question 26

most common organ of deposition

Answer 26

kidney- nephrotic syndrome (proteinuria)

Question 27

type II diabetes

Answer 27

deposition of amylin in islets of pancreas