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HDT 3.1 > Autoinflammatory disease > Flashcards

Autoinflammatory disease Flashcards

1
Q

what is autoinflammation?

A

mutations in cells or molecules involved in
innate immunity at disease-prone sites

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2
Q

what is autoimmunity?

A

mutations associated with cells and molecules involved in adaptive immune responses

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3
Q

why are polygenic mutations harder to treat?

A

as they have multiple mutations to many different geners

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4
Q

what are some examples of polygenic mutations?

A

crohns disease
rheumatoid arthritis
multiple sclerosis

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5
Q

what is monogenic autoinflammatory diseases?

A

seemingly unprovoked attacks of inflammation

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6
Q

what occurs when you have monogenic autoinflammatory diseases?

A

absence of high-tier autoantibodies or antigen-specific T cells
in born errors of the innate immune system

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7
Q

what is CAPS?

A

cryopyrin associated periodic syndrome

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8
Q

what is the spectrum of CAPS?

A

Familial cold autoinflammatory syndrome(FCAS) - Rash, conjuctivitus

Muckle wall syndrome (MWS) - Urticarial rash

NOMID/CINCA - Sporadic, deafness, destructive artheritis

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9
Q

why are autoinflammatory diseases also named periodic fever syndrome?

A

as the individual will experience fluctuations as the diseased state flares up

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10
Q

what are NLR?

A

intracellular receptors

N-terminal effector domain - CARD n Prying domain

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11
Q

what is ASC?

A

CARD and pyrin domains

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12
Q

what happens when procaspase-1 is in contact with NLR?

A

cleaved into caspase-1, which cleaves pro-IL-1B and pro-IL-18 into IL-1B and IL-18

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13
Q

what happens when caspase-1 is formed ?

A

cleaves pro-IL-1b and pro-IL-18 into IL-1b and IL-18

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14
Q

what are inflammasomes?

A

innate immune system receptors/sensors that regulate activation of caspase-1 and induce inflammation

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15
Q

what are some examples of inflammasomes?

A

Four key inflammasomes, namely NLRP1, NLRP3, NLRC4, and AIM2

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16
Q

what happens when inflammasomes have been activated?

A

processing and secretion of inflammatory cytokines, including IL-1β and IL-18

17
Q

what happens to people with CAPS responding to lipopolysaccharide (LPS)?

A

react more by producing more IL-1B to low concentrations of LPS (increasing sensitivity to LPS) and induces an immune response as the IL-1b levels are elevated

18
Q

what does IL-1B act on?

A
19
Q

what are the examples of anti-IL-1b therapies?

A
20
Q

what are NOD sensors/receptors?

A

stimulate antimicrobial peptide (AMP) and Type 1 interferon production (IFNb)

21
Q

what is blau syndrome?

A

Blau syndrome is a rare, autosomal dominant disease characterized by granulomatous skin/rash, eye and joint inflammation-arthritis.

mutated NOD2 sensor

22
Q

how are misfolded proteins normally treated?

A

eliminated by degradation in the endoplasmic reticulum

23
Q

how does unfolded protein response (UPR) react?

A

a homeostatic response balancing the cells folding capacity

24
Q

what happens if there is an imbalance in the UPR?

A

leads to endoplasmic reticulum stress and causes an increase in the unfolded proteins

25
Q

why is an increase in UPR a problem?

A

as this means there can be inhibition of translation causing cell death

and misfolded proteins accumulate extracellularly which induces an IL-1B production

26
Q

how can physiological conditions alter the ERAD and protein folding?

A
27
Q

what does ER stress induce?

A
28
Q

what are the differences between autoinflammation and autoimmunity?

A
29
Q

what is CAPS associated with?

A

elevated c-reactive protein
serum amyloid-a

30
Q

what do A20 and OTULIN regulate? and how?

A

NF-kB and Cell death signalling
negatively

31
Q

what does a decrease expression in A20 and OTULIN do?

A

activate NF-kB = release pro-inflame cytokines

32
Q

what does an activated NF-kb do?

A

release pro-inflam cytokines

HDT 3.1 (13 decks)

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