Practical 6: Agglutination and Primary Immunodeficiency Flashcards

1
Q

What antibody functions are associated with the Fab fragment?

A

Antigen binding

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2
Q

What antibody functions are associated with the Fc fragment

A

Complement binding

Binding to Fc receptors

Placental transfer

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3
Q

In antibody dimers, pentamers etc, what holds the monomers in place?

A

Disulfide bonds

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4
Q

What are the 6 functions of antibodies

A

Activates complement

Triggers mast cell degranulation

Activates antibody-dependent cellular activity

Causes antigen clumping and inactivation of bacterial toxins

Acts as opsonins

Activates B lymphocytes

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5
Q

Antibodies are aglutanins, what does this mean?

A

They can clump antibodies together

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6
Q

What does the constant domain determine?

A

The classification of the antibody

The functions of the antibody

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7
Q

Why is IgM a very effective agglutinin?

A

It is a pentamer
Valency of ten

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8
Q

What is haemagglutination?

A

The agglutination of red blood cells

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9
Q

What are the two main uses of haemoagglutination?

A

Blood typing

Viral quantification

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10
Q

How is blood grouping/typing carried out?
(2)

A

Use of anti sera (antibodies against certain blood groups)

e.g. agglutination will occur if the anti-sera binds to the erythrocyte i.e. if the anti sera is for the antigen expressed on the rbc

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11
Q

What is rheumatoid factor?
(2)

A

The antibody that was first found in rheumatoid arthritis

An antibody against the Fc portion of IgG

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12
Q

How does rheumatoid factor cause arthiris?

A

This factor binds to the Fc portion of IgG to form immune complexes which contribute to the disease

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13
Q

How is a doubling dilution carried out?

A

Transfer 0.5ml from first tube into the next, then transfer 0.5ml from second tube into third etc

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14
Q

What is your control in this experiment?

A

Only saline - no antibodies (but add the rbcs)
Negative control

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15
Q

What should you not do in this experiment?

A

Don’t vortex - this will lyse the rbcs

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16
Q

What does a tight pellet mean?

A

High agglutination

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17
Q

What happens to the pellet as the samples get more dilute?

A

The pellet diffuses more - lower agglutination

18
Q

What is a primary immunodeficiency?

A

Disorders that are caused by an inherited flaw in the immune system that increases the susceptibility to infections

19
Q

What are secondary or acquired immmune deficiency disease caused by?

A

Infectious, chemical or radiological agents

20
Q

Write about chronic granulomatosis disease
(3)

A

The X-linked form is most common
Causes granulomas (bacteria are ingested by phagocytes but not digested quick enough)

The enzyme that makes the needed chemicals is missing

21
Q

What enzyme is involved in chronic granulomatosis disease?

A

NADPH oxidase

22
Q

What is NADPH oxidase?
(2)

A

A complex, multisubunit enzyme

Its coded by a gene on the X chromosome

23
Q

What are the subunits of NADPH oxidase?
(5)

A

p40phox
p47phox
p67phox
p22phox

gp91phox

24
Q

What subunit is most important to NADPH oxidase?

A

gp91phox

25
Q

What is phox?

A

Phagosome oxidase

26
Q

What happens in a respiratory burst?

A

The cell takes in oxygen

27
Q

Explain how NADPH oxidase works in a healthy cell vs CGD
(2)

A

After respiratory burst all the subunits of NADPH Oxidase should come together to react on the oxygen and form reactive oxygen species to kill the pathogens

In CGP there is no/no active gp91phox => no reactive oxygen species can be formed

28
Q

Give two examples of reactive oxygen species

A

Hydrogen peroxide

Super oxide anion

29
Q

Write about leucocyte adhesion deficiency
(3)

A

Leucocytes don’t express CD18, no adhesion molecules are expressed, they can’t adhere to endothelial cells and get into imflammed tissues

This causes gingivitis (inflammation of the gums)

Causes omphalitis (inflammation of the umbilical cord)

30
Q

What is inflammation of the umbilical cord called?

A

Omphalitis

31
Q

What are the four types of adhesion molecules?

A

Vascular addressin (CD34) (Mucin-like CAM)

Selectin (L-selectin)

Integrin (LFA-1)

Immunoglobulin-like molecule (ICAM-1)

32
Q

What are the steps in the adhesion cascade?
(5)

A

Capture

Rolling

Activation

Adhesion

Transmigration

33
Q

How many types of LAD are there?

A

Three types LAD I, II and III

34
Q

What causes LAD I

A

Lack of integrins: CD18

Deficiency in B2 integrins

35
Q

What are the symptoms of LAD I
(6)

A

Leukocytosis

Bacterial infections

Sepsis

Gingivitis

Omphalitis

Bleeding

36
Q

What are the symptoms of LAD II?
(6)

A

Leukocytosis

Bacterial infections

Sepsis

Gingivitis

Omphalitis

Bleeding

37
Q

What causes LAD II?

A

Integrin activation

38
Q

What causes LAD III?

A

Selectins

39
Q

What are the symptoms of LAD III?
(4)

A

Mental retardation

Microcephaly

Dwarfism

Recurrent infections with neutrophils

40
Q

What is XLA?

A

X-linked agammaglobulineamia

41
Q

What causes XLA?

A

A deficiency of B cells (BtK)