Exam 2 Flashcards

1
Q

T cells recognize antigen when the antigen

A

forms a complex with membrane-bound MHC molecules on another host-derived cell

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2
Q

If viewing the three-dimensional structure of a T-cell receptor from the side, with the T-cell membrane at the bottom and the receptor pointing upwards, which of the following is inconsistent with experimental data?
a. The highly variable CDR loops are located across the top surface.
b. The membrane-proximal domains consist of Cα and Cβ.
c. The portion that makes physical contact with the ligand comprises Vβ and Cβ, the domains farthest
fromtheT-cellmembrane.
d. The transmembrane regions span the plasma membrane of the T cell.
e. The cytoplasmic tails of the T-cell receptor α and β chains are very short

A

C) The portion that makes physical contact with the ligand comprises Vβ and Cβ, the domains farthest
fromtheT-cellmembrane.

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3
Q

Unlike B cells, T cells do not engage in any of the following processes except _______________.

a. alternative splicing to produce a secreted form of the T-cell receptor
b. alternative splicing to produce different isoforms of the T-cell receptor
c. isotype switching
d. somatic hypermutation
e. somatic recombination

A

E) Somatic Recombination

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4
Q

Unlike the C regions of immunoglobulin heavy-chain loci, the C regions of the T-cell receptor β-chain loci ______.

A

Functionally similar

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5
Q

Which of the following statements regarding Omenn syndrome is incorrect?
a. A bright red, scaly rash is due to a chronic inflammatory condition.
b. Affected individuals are susceptible to infections with opportunistic pathogens.
c. It is invariably fatal unless the immune system is rendered competent through a bone marrow
transplant.
d. It is the consequence of complete loss of RAG function.
e. There is a deficiency of functional B and T cells.
f. It is associated with missense mutations of RAG genes.

A

D) It is the consequence of the complete loss of RAG function.

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6
Q

All of the following statements regarding γ:δ T cells are correct except ___________.

a. they are more abundant in tissue than in the circulation
b. the δ chain is the counterpart to the β chain in α:β T-cell receptors because it contains V, D, and J segments in the variable region
c. they share some properties with NK cells
d. activation is not always dependent on recognition of a peptide:MHC molecule complex
e. expression on the cell surface is not dependent on the CD3 complex

A

E) Expression on the cell surface is not dependent on the CD3 complex.

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7
Q

During T-cell receptor _____-gene rearrangement, two D segments may be used in the final rearranged gene sequence, thereby increasing overall variability of this chain.

a. α
b. β
c. γ
d. δ
e. ε.

A

D) δ

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8
Q

The degradation of pathogen proteins into smaller fragments called peptides is a process commonly referred to as

A

Antigen processing

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9
Q

The primary reason for transplant rejections is due to differences in _____ between donor and recipient.

A

MHC Molecules

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10
Q

When describing the various components of the vesicular system, which of the following is not included?

a. Nucleus
b. Golgi apparatus
c. endoplasmic reticulum
d. exocytic vesicles
e. lysosomes

A

Nucleus

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11
Q

Which of the following is not a characteristic of immunoproteasomes?
a. They make up about 1% of cellular protein.
b. They consist of four rings of seven polypeptide subunits that exist in alternative forms.
c. They are produced in response to IFN-γ produced during innate immune responses.
d. They produce a higher proportion of peptides containing acidic amino acids at the carboxy terminus
compared with constitutive proteasomes.
e. They contain 20S proteasome-activation complexes on the caps.

A

D) They produce a higher proportion of peptides containing acidic amino acids at the carboxy-terminus compared with constitutive proteasome.

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12
Q

Identify which of the following statements is true regarding the transporter associated with antigen processing (TAP).
2
a. TAP is a homodimer composed of two identical subunits.
b. TAP transports proteasome-derived peptides from the cytosol directly to the lumen of the Golgi
apparatus.
c. TAP is an ATP-dependent, membrane-bound transporter.
d. Peptides transported by TAP bind preferentially to MHC class II molecules.
e. TAP deficiency causes a type of bare lymphocytes syndrome resulting in severely depleted levels of
MHC class II molecules on the surface of antigen-presenting cells.

A

C) TAP is an ATP-dependent, membrane-bound transporter.

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13
Q

All of the following are included in the peptide-loading complex except _____________.

a. tapasin
b. calnexin
c. calreticulin
d. ERp57
e. β2-microglobulin

A

B) Calnexin

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14
Q
Which of the following best describes the function of tapasin?
a. Tapasin is an antagonist of HLA-DM and causes more significant increases in MHC class I than MHC
class II on the cell surface.
b. Tapasin is a lectin that binds to sugar residues on MHC class I molecules, T-cell receptors, and
immunoglobulins and retains them in the ER until their subunits have adopted the correct
conformation.
c. Tapasin is a thiol-reductase that protects the disulfide bonds of MHC class I molecules.
d. Tapasin participates in peptide editing by trimming the amino terminus of peptides to ensure that
the fit between peptide and MHC class II molecules is appropriate.
e. Tapasin is a bridging protein that binds to both TAP and MHC class I molecules and facilitates the selection of peptides that bind tightly to MHC class I molecules.
A

E) Tapasin is a bridging protein that binds to both TAP and MHC class I molecules and facilitates the selection of peptides that bind tightly to MHC class I molecules.

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15
Q

Which of the following characteristics is common to both T-cell receptors and immunoglobulins?

a. Somatic recombination of V, D, and J segments is responsible for the diversity of antigen-binding sites.
b. Somatic hypermutation changes the affinity of antigen-binding sites and contributes to further diversification.
c. Class switching enables a change in effector function.
d. The antigen receptor is composed of two identical heavy chains and two identical light chains.
e. Carbohydrate, lipid, and protein antigens are recognized and stimulate a response.

A

A) somatic recombination of V,D, and J segments is responsible for the diversity of antigen-binding sites.

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16
Q

The antigen-recognition site of T-cell receptors is formed by the association of which of the following domains?

a. Vα and Cα
b. VβandCβ
c. CαandCβ
d. VαandCβ
e. Vα and Vβ

A

E) Vα and Vβ

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17
Q

How many complementarity-determining regions contribute to the antigen-binding site in an intact T-cell receptor?

A

6

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18
Q

IgG possesses _______ binding sites for antigen, and the T-cell receptor possesses _______ binding sites for antigen:

A

2;1

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19
Q

In B cells, transport of immunoglobulin to the membrane is dependent on association with two invariant proteins, Igα and Igβ. Which of the following invariant proteins provide this function for the T-cell receptor in T cells?

a. CD3γ
b. CD3δ
c. CD3ε
d. ζ
e. All of the above

A

E) All of the above

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20
Q

Which of the following is not a characteristic of native antigen recognized by T cells?

a. peptides ranging between 8 and 25 amino acids in length
b. not requiring degradation for recognition
c. amino acid sequences not found in host proteins
d. primary, and not secondary, structure of protein
e. binding to major histocompatibility complex molecules on the surface of antigen-presenting cells.

A

B) not requiring degradation for recognition

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21
Q

Which of the following statements regarding CD8 T cells is incorrect?

a. When activated, CD8 T cells in turn activate B cells.
b. CD8 is also known as the CD8 T-cell co-receptor.
c. CD8 binds to MHC molecules at a site distinct from that bound by the T-cell receptor.
d. CD8 T cells kill pathogen-infected cells by inducing apoptosis.
e. CD8 T cells are MHC class I-restricted.

A

A) When activated, CD8 T-cels in turn activate B cells

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22
Q

Antigen processing involves the breakdown of protein antigens and the subsequent association of peptide fragments on the surface of antigen-presenting cells with ________________.

A

MHC class I or MHC class II molecules

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23
Q

Which of the following statements regarding T-cell receptor recognition of antigen is correct?
a. α:β T-cell receptors recognize antigen only as a peptide bound to an MHC molecule.
b. αβ T-cell receptors recognize antigens in their native form.
c. α:β T-cell receptors, like B-cell immunoglobulins, can recognize carbohydrate, lipid, and protein
antigens.
4
d. Antigen processing occurs in extracellular spaces.
e. Like α:β T cells, γ:δ T cells are also restricted to the recognition of antigens presented by MHC
molecules.

A

A) α:β T-cell receptors recognize antigen only as a peptide bound to an MHC molecule.

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24
Q
  1. Which of the following describes a ligand for an α:β T-cell receptor?
    a. carbohydrate:MHC complex
    b. lipid:MHC complex
    c. peptide:MHC complex
    d. all of the above
    e. none of the above
A

C) peptide:MHC complex

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25
Q

MHC class II molecules are made up of two chains called _______, whose function is to bind peptides and present them to _______ T cells.

A

alpha (α) and beta (β); CD4

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26
Q

The CDR3 loops of the T-cell receptor contact the _______.

A

side chains of amino acids in the middle of the peptide.

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27
Q

MHC molecules have promiscuous binding specificity. This means that

A

a particular MHC molecule has the potential to bind to different peptides.

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28
Q

T-cell receptors interact not only with peptide anchored in the peptide-binding groove of MHC molecules, but also with ____________________.

A

variable amino acid residues on α helices of the MHC molecule

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29
Q

Cross-priming of the immune response occurs when ______________________________.

A

viral antigens are presented by MHC class I molecules on the surface of a cell that is not actually infected by that particular virus, or peptides of nuclear or cytosolic proteins are presented by MHC class II molecules.

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30
Q

In reference to the interaction between T-cell receptors and their corresponding ligands, which of the following statements is correct?

a. The organization of the T-cell receptor antigen-binding site is distinct from the antigen-binding site of immunoglobulins.
b. The orientation between T-cell receptors and MHC class I molecules is different from that of MHC class II molecules.
c. The CDR3 loops of the T-cell receptor α and β chains form the periphery of the binding site making contact with the α helices of the MHC molecule.
d. The most variable part of the T-cell receptor is composed of the CD3 loops of both the α and β
chains.
e. All of the above statements are correct.

A

D) The most variable part of the T-cell receptor is composed of the CD3 loops of both the ∂ and ß chains.

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31
Q

The diversity of MHC class I and II genes is due to ___________________.

A

the existence of many similar genes encoding MHC molecules in the genome and extensive
polymorphism at many of the alleles

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32
Q

The combination of all HLA class I and class II allotypes that an individual expresses is referred to as their ____________.

A

HLA type

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33
Q

All of the following are oligomorphic except ___________________.

a. HLA-G α chain
b. HLA-DO β chain
c. HLA-DQ β chain
d. HLA-A α chain
e. HLA-DR α chain

A

C) HLA-DQ β chain

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34
Q

All of the following are highly polymorphic except _____________.

a. HLA-A α chain
b. HLA-DO α chain
c. HLA-B α chain
d. HLA-DR β chain
e. HLA-C α chain

A

B) HLA-DO α chain

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35
Q
Of the following HLA α-chain loci, which one exhibits the highest degree of polymorphism?
6
a. HLA-A
b. HLA-B 
c. HLA-C
d. HLA-DP
e. HLA-DR
A

B) HLA-B

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36
Q

Which of the following is/are not encoded on chromosome 6 in the HLA complex?

a. β2-microglobulin and invariant chain
b. HLA-G α chain
c. TAP-1
d. tapasin
e. HLA-DR α chain

A

A) β2-microglobulin and invariant chain

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37
Q

The _____ refers to the complete set of HLA alleles that a person possesses on a particular chromosome 6.

A

Haplotype

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38
Q

Peptides that bind to a particular MHC isoform usually have either the same or chemically similar amino acids at two to three key positions that hold the peptide tightly in the peptide-binding groove of the MHC molecule. These amino acids are called _____ and the combination of these key residues is known as its _________________.

A

anchor residues; peptide-binding motif

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39
Q

Directional selection is best described as ______________________________.

A

selected alleles increase in frequency in a population

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40
Q

The role of the CD3 proteins and ζ chain on the surface of the cell is to ___________________.

A

transduce signals to the interior of the T cell

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41
Q

The immunological consequence of severe combined immunodeficiency disease (SCID) caused by a genetic defect in either RAG-1 or RAG-2 genes is _____________________.

A

lack of somatic recombination in T-cell receptor and immunoglobulin gene loci

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42
Q

Which of the following removes CLIP from MHC class II molecules?

a. HLA-DM correct
b. HLA-DO
c. HLA-DP
d. HLA-DQ
e. HLA-DR

A

E) HLA-DR

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43
Q

CD8 T-cell subpopulations are specialized to combat _______ pathogens, whereas CD4 T-cell subpopulations are specialized to combat _______ pathogens.

A

intracellular; extracellular

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44
Q

Which of the following describes the sequence of events involved in processing of peptides that will be presented as antigen with MHC class I?

A

proteasome →TAP1/2 →MHC class I →endoplasmic reticulum →plasma membrane

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45
Q

One type of bare lymphocyte syndrome is caused by a genetic defect in MHC class II transactivator (CIITA), which results in the inability to synthesize MHC class II and display it on the cell surface. The consequence of this would be that ____________________________.

A

CD4 T-cells cannot function

46
Q

Which of the following describes the sequence of events involved in the processing of peptides that will be presented as antigen with MHC class II?

A

endocytosis →protease activity →removal of CLIP from MHC class II →binding of peptide to MHC class II →plasma membrane

47
Q

Which of the following cell types does not express MHC class I?

a. erythrocyte
b. hepatocyte
c. lymphocyte
d. dendritic cell
e. neutrophil

A

A) Erythrocyte

48
Q

Which of the following cell types is not considered a professional antigen-presenting cell?

a. macrophage
b. neutrophil
c. B cell
d. dendritic cell
e. all of the above are professional antigen-presenting cells

A

B) Neutrophil

49
Q

Which of the following is mismatched?
a. peptide-binding motif: combination of anchor residues in a peptide capable of binding a particular
MHC haplotype
b. MHC restriction: specificity of T-cell receptor for a particular peptide:MHC molecule complex
c. balancing selection: maintenance of variety of MHC isoforms in a population
d. directional selection: replacement of older MHC isoforms with newer variants
e. interallelic conversion: recombination between two different genes in the same family

A

E) interallelic conversion: recombination between two different genes in the same family

50
Q

Which is the most likely reason that HIV-infected people with heterozygous HLA loci have a delayed progression to AIDS compared with patients who are homozygous at one or more HLA loci?

a. The greater number of HLA alleles provides a wider variety of HLA molecules for presenting HIV- derived peptides to CD8 T cells even if HIV mutates during the course of infection.
b. Heterozygotes have more opportunity for interallelic conversion and can therefore express larger numbers of MHC alleles.
c. Directional selection mechanisms favor heterozygotes and provide selective advantage to pathogen exposure.
d. As heterozygosity increases, so does the concentration of alloantibodies in the serum, some of which cross-react with and neutralize HIV

A

A) The greater number of HLA alleles provides a wider variety of HLA molecules for presenting HIV- derived peptides to CD8 T cells even if HIV mutates during the course of infection.

51
Q

Which of the following cell-surface markers differentiates hematopoietic stem cells from other cell constituents in the bone marrow?

a. pre-B-cell receptor
b. BAFF receptor
c. CD34
d. CD4
e. membrane-bound stem-cell factor (SCF)

A

CD34

52
Q

Which of the following is characteristic of a large pre-B cell?

a. VDJ is successfully rearranged and mu heavy chain is made.
b. V–J is rearranging at the light-chain locus.
c. mu heavy chain and lambda or kappa light chain is made.
d. V is rearranging to DJ at the heavy-chain locus.
e. D–J is rearranging at the heavy-chain locus

A

A) VDJ is successfully rearranged and mu heavy chain is made.

53
Q

Which of the following statements is correct?

a. The κ light-chain genes rearrange before the heavy-chain genes.
b. The κ light-chain genes rearrange before the λ light-chain genes.
c. The λ light-chain genes rearrange before the heavy-chain genes.
d. The λ light-chain genes rearrange before the κ light-chain genes.
e. The μ heavy-chain genes rearrange first and then the λ light-chain genes rearrange

A

B) The κ light-chain genes rearrange before the λ light-chain genes.

54
Q

Immature B cells develop into B cells in the

A

Secondary Lymphoid organs

55
Q

Large pre-B cells are characterized by which of the following?

a. They do not express CD19 at the cell surface.
b. Rearrangement of light-chain genes commences.
c. Nonproductive rearrangement of both heavy-chain loci has already occurred.
d. Allelic exclusion of the immunoglobulin light-chain loci has already occurred.
e. The mu chain is assembled with VpreB-lambda5.

A

E) the Mu chain is assembled with VpreB-lambda5.

56
Q

All hematopoietic stem cells express

A

CD34

57
Q

The latest stages of late pro-B-cell development are recognized by the association of a surrogate light chain with a mu chain. The surrogate light chain is composed of _______.

A

VpreB and lambda5

58
Q

Which of the following is not paired with its correct complement?
a. N nucleotides: more abundant in rearranged heavy-chain genes than in rearranged light-chain
genes
b. second checkpoint in B-cell development: assembly of a functional B-cell receptor
c. receptor editing: exchange of light chain for one that is not self-reactive
d. first checkpoint in B-cell development: selection by the pre-B-cell receptor
e. large pre-B-cell stage: constitutive expression of RAG-1 and RAG-2 proteins.

A

e. large pre-B-cell stage: constitutive expression of RAG-1 and RAG-2 proteins.

59
Q

Which of the following would occur after the production of a functional μ chain as a pre-B-cell receptor?

a. RAG proteins are degraded.
b. The chromatin structure of the heavy-chain locus is reorganized to prevent gene rearrangement.
c. Transcription of the RAG1 and RAG2 genes ceases.
d. There is allelic exclusion of a second mu chain.
e. All of the above would occur.

A

E) All of the above would occur

60
Q

An important advantage of having two gene loci (kappa and lambda) for the light chain is ________.

A

that the likelihood of a successful rearrangement of light-chain genes increases

61
Q

A defect in which of the following proteins blocks B-cell development at the pre-B-cell stage, resulting in almost no circulating antibodies in individuals with this defect?

a. IL-7 receptor
b. terminal deoxynucleotidyltransferase (TdT)
c. Pax-5
d. Bruton’s tyrosine kinase (Btk)
e. CD19

A

D) Bruton’s tyrosine kinase (Btk)

62
Q

The consequence of allelic exclusion at the immunoglobulin loci ensures that _____.

A

B cells express antigen receptors of a single specificity and homogeneous B-cell receptors bind
more effectively to antigen

63
Q

A developing B cell unable to generate a productive rearrangement on any of the four light-chain loci will undergo _____________.

A

Apoptosis

64
Q

All of the following participate in signal transduction in developing B cells except:

a. terminal deoxynucleotidyl transferase (TdT)
b. FLT3
c. CD19
d. Igα and Igβ
e. Bruton’s tyrosine kinase (Btk)

A

A) Terminal deoxynucleotidyl transferase (TdT)

65
Q

Negative selection of developing B cells ensures that ___________.

A

B-cell receptors that bind to normal constituents of the body do not emerge

66
Q

Receptor editing occurs _____.

A

in the bone marrow to establish self-tolerance of the B-cell repertoire.

67
Q

Which of the following statements about the IgD made by B cells of upper respiratory mucosa is not true?

a. These antibodies bind to airborne bacteria such as Haemophilus influenzae.
b. lambda light chains are used almost exclusively by these IgD antibodies.
c. Two-thirds of these IgD antibodies possess kappa light chains.
d. These IgD antibodies recruit basophils and induce the secretion of antibacterial peptides.
e. kappa light chains are used almost exclusively by these IgD antibodies.

A

c. Two-thirds of these IgD antibodies possess kappa light chains.

68
Q

All of the following are associated with the development of Burkitt’s lymphoma except:
a. The expression of Myc protein is perturbed.
b. A chromosomal translocation involving a proto-oncogene and an immunoglobulin gene occurs.
c. Overproduction of the Bcl-2 protein prolongs the lifetime of B-lineage cells.
d. Cell division restraints on mutated B cells are lifted.
e. In addition to a chromosomal translocation event, mutations elsewhere in the genome are usually
involved.

A

c. Overproduction of the Bcl-2 protein prolongs the lifetime of B-lineage cells.

69
Q

Which of the following is a characteristic of B-2 cells?

a. They are sometimes referred to as CD5 B cells.
b. They comprise only 5% of the B-cell repertoire.
c. In adults, they are renewed by cell division in the peripheral circulation.
d. They are located primarily in secondary lymphoid organs.
e. They are not dependent on T helper cells for activation.

A

d. They are located primarily in secondary lymphoid organs.

70
Q

Identify the mismatched pair of chemokine and the cells that secrete it.

a. CCL19: lymph-node dendritic cells
b. CXCL13: follicular dendritic cells
c. CCL21: stromal cells of secondary lymphoid tissues
d. All of the above are correctly matched.
e. None of the above is correctly matched.

A

D) All of the above are correctly matched.

71
Q

Plasma cells have all of the properties listed except ______________.
a. they rapidly proliferate in secondary lymphoid follicles
b. they secrete antibody and they are terminally differentiated B cells
c. they no longer express MHC class II molecules
d. they cease expressing membrane-bound immunoglobulin
e. differentiation into plasma cells occurs after migration from germinal centers to other sites in
lymphoid tissue and bone marrow

A

a. they rapidly proliferate in secondary lymphoid follicles

72
Q

All of the following events occur within germinal centers except:

a. production of memory B cells
b. isotype switching
c. centroblasts arise from activated B cells
d. B cells are activated by CD4 helper T cells
e. affinity maturation

A

d. B cells are activated by CD4 helper T cells

73
Q

In which location would plasma cells not be present?

a. bone marrow
b. afferent lymphatic vessels
c. medullary cords of lymph nodes
d. lamina propria of gut-associated lymphoid tissues
e. efferent lymphatic vessels

A

b. afferent lymphatic vessels

74
Q

mmunological tolerance in the B-cell repertoire is called _______ tolerance when it develops in primary lymphoid organs, and _______ tolerance when it is induced outside the bone marrow.

A

central; peripheral

75
Q

When producing monoclonal antibodies, why is it important to use as a fusion partner a myeloma cell that is unable to produce its own immunoglobulin?

A

To ensure that the antibodies are homogeneous and able to make strong bivalent attachments to
multivalent antigens.

76
Q

The proto-oncogene _______ is associated with the development of Burkitt’s lymphoma.

A

Myc

77
Q

Which of the following characterizes the B-1 cells that develop prenatally?
a. They lack N nucleotides.
b. They possess polyspecificity for bacterial polysaccharide antigens.
c. They arise early in embryonic development preceding the development of the majority subset of B
cells.
d. They have little or no IgD on the cell surface.
e. All of the above.

A

E) All of the Above

78
Q

What is the fate of an immature B cell that encounters and has specificity for self antigen?

A

Continued rearrangement of light-chain genes.

79
Q

Which of the following pertains to the fate of immature B cells that have specificity for univalent self antigens?

a. The cells acquire a state of unresponsiveness called anergy.
b. IgD is retained in the cytosol.
c. IgD on the cell surface activate the B cell when bound to self antigen.
d. The cells have a much longer life-span than mature B cells.
e. The cells die by apoptosis.

A

a. The cells acquire a state of unresponsiveness called anergy.

80
Q

The circulatory route through a lymphoid tissue for both immature B cells and mature B cells that do not encounter specific antigen is:

A

bloodstream –> HEV of lymphoid cortex –> primary lymphoid follicle –> efferent lymphatic vessel

81
Q

In which of the following ways does the developmental pathway of alpha:beta T cells differ from that of B cells? (Select all that apply.)
a. Their antigen receptors are derived from gene rearrangement processes.
b. When the first chain of the antigen receptor is produced it combines with a surrogate chain.
c. Cells bearing self-reactive antigen receptors undergo apoptosis.
d. MHC molecules are required to facilitate progression through the developmental pathway and T
cells do not rearrange their antigen-receptor genes in the bone marrow.
e. T cells rearrange their antigen-receptor genes in the bone marrow.

A

d. MHC molecules are required to facilitate progression through the developmental pathway and T
cells do not rearrange their antigen-receptor genes in the bone marrow.

82
Q

Which of the following cell-surface glycoproteins is characteristic of stem cells, but stops being expressed when a cell has committed to the T-cell developmental pathway?

a. CD2
b. CD3
c. CD25
d. CD34
e. MHC class II

A

D) CD34

83
Q

Which of the following processes is not dependent on an interaction involving MHC class I or class II molecules?

a. positive selection of alpha:beta T cells
b. intracellular signaling by pre-T-cell receptors and positive selection of gamma:delta T cells
c. negative selection of alpha:beta T cells
d. peripheral activation of mature naive T cells
e. None of the above.

A

B) intracellular signaling by pre-T-cell receptors and positive selection of gamma:delta T cells

84
Q

If a double-negative thymocyte has just completed a productive beta-chain gene rearrangement, which of the following describes the immediate next step in the development of this thymocyte?

a. A pre-T-cell receptor is assembled as a superdimer.
b. Rearrangement of gamma- and delta-chain genes commences.
c. Expression levels of RAG-1 and RAG-2 are elevated.
d. The linked delta-chain genes are eliminated.
e. This cell will inevitably differentiate into a committed gamma:delta T cell.

A

a. A pre-T-cell receptor is assembled as a superdimer.

85
Q

All of the following cell-surface glycoproteins are expressed by double-negative thymocytes undergoing maturation in the thymus except _____.

a. CD2
b. CD5
c. CD127 (IL-7 receptor)
d. CD34 and CD4
e. CD1A

A

d. CD34 and CD4

86
Q

_____ is a T-cell-specific adhesion molecule expressed before the expression of a functional T- cell receptor while the thymocytes are still in their double-negative stage of development.

A

CD2

87
Q

Which of the following is mismatched:

a. double-negative CD3– thymocytes: cortico-medullary junction
b. double-negative CD3– thymocytes: subcapsular zone
c. double-positive CD3+ thymocytes: cortico-medullary junction
d. cortical epithelial cells: subcapsular regions
e. dendritic cells: cortico-medullary junction.

A

a. double-negative CD3– thymocytes: cortico-medullary junction

88
Q

Which of the following statements about Notch 1 is incorrect?
correct
a. Notch 1 is expressed on thymic epithelial cells, and in the absence of Notch 1 expression, T cells can complete their differentiation.
b. Notch 1 is to T-cell development as Pax-5 is to B-cell development.
c. Notch 1 contains two distinct domains, one of which is proteolytically cleaved and becomes a
transcription factor in the nucleus.
d. The extracellular domain of Notch 1 must interact with a ligand on thymic epithelium to initiate
cleavage and separation of the Notch 1 extracellular and intracellular domains.

A

a. Notch 1 is expressed on thymic epithelial cells, and in the absence of Notch 1 expression, T cells can complete their differentiation.

89
Q

Which of the following is the first stage of T-cell receptor gene rearrangement in alpha:beta T cells?

a. V-alpha –> D-alpha
b. D-alpha –> J-alpha
c. V-beta –> D-beta
d. D-beta –> J-beta
e. V-alpha –> J-alpha

A

d. D-beta –> J-beta

90
Q

Which of the following is the first T-cell receptor complex containing the β chain to reach the cell surface during the development of T lymphocytes?

a. gamma:beta:CD3
b. beta:CD3
c. alpha:beta:CD3
d. beta:CD44
e. pTalpha:beta:CD3

A

e. pTalpha:beta:CD3

91
Q

Genetic deficiencies in all of the following would impair the development of a fully functional T- cell repertoire except

a. RAG-1 or RAG-2
b. Notch1
c. Pax-5
d. IL-7 receptor (CD127)
e. TAP-1 or TAP-2

A

c. Pax-5

92
Q

There are many parallels between the development of B cells and T cells. Identify the incorrectly matched counterpart in B cells (left) versus T cells (right).
a. VpreBlambda5: pTalpha
b. Igalpha/Igbeta:CD3
c. Pax-5: FoxP3
d. multiple kappa and lambda light-chain gene rearrangements: multiple alpha-chain gene
rearrangements.
e. Pax-5: CD3

A

c. Pax-5: FoxP3

93
Q

_______ of thymocytes is necessary to produce a T-cell repertoire capable of interacting with self-MHC molecules.

A

Positive Selection

94
Q

Which of the following statements is false of a T cell that expresses two α chains (and thus two different T-cell receptors) as a result of ineffective allelic exclusion of the α chain during rearrangement?

a. Engaging either of the T-cell receptors on MHC molecules of the thymic epithelium will result in positive selection.
b. One of the T-cell receptors will be functional while the other will most probably be non- functional.
c. If either T-cell receptor binds strongly to self-peptides presented by self-MHC molecules, the thymocyte will be negatively selected.
d. One of the T-cell receptors may be autoreactive but escape negative selection because its peptide antigen is present in tissues other than the thymus.
e. Subsequent gene rearrangements may give rise to a gamma:delta T-cell receptor.

A

e. Subsequent gene rearrangements may give rise to a gamma:delta T-cell receptor.

95
Q

Once a thymocyte has productively rearranged a beta-chain gene, which of these events cannot occur subsequently?
a. beta binds to pTalpha and is expressed on the cell surface with the CD3 complex and zeta chain.
b. Rearrangement of beta-, gamma- and delta-chain genes ceases as a result of the suppression of
expression of RAG-1 and RAG-2.
c. The pre-T cell proliferates and produces a clone of cells all expressing an identical beta chain.
d. Expression of CD34 and CD2 gives rise to double-positive thymocytes.
e. Alpha-, gamma-, delta-chain loci rearrange simultaneously.

A

d. Expression of CD34 and CD2 gives rise to double-positive thymocytes.

96
Q
Which of the following statements regarding positive selection is correct?
a. All subsets of developing T cells undergo positive selection before export to the peripheral
circulation.
b. T-cell receptor editing is linked to the process of positive selection. 
c. Positive selection results in the production of T cells bearing T-cell receptors that have the capacity to interact with all allotypes of MHC class I and class II molecules, and not just those of the individual.
d. Positive selection ensures that autoreactive T cells are rendered non-responsive.
e. If there is a genetic defect in AIRE, then T-cell development is arrested as positive selection
commences.
A

b. T-cell receptor editing is linked to the process of positive selection.

97
Q

Thymocytes that are not positively selected

A

make up about 98% of developing thymocytes and die by apoptosis in the thymic

98
Q

If the process of positive selection did not occur, then ______.

A

only a very small percentage of circulating T lymphocytes would be able to become activated.

99
Q

Immediately after positive selection __________.

A

RAG proteins are degraded and are no longer synthesized

100
Q

Allelic exclusion occurs for all of the following except _____________.

a. T-cell receptor alpha genes
b. T-cell receptor beta genes
c. B-cell receptor heavy-chain genes
d. B-cell receptor kappa-chain genes
e. B-cell receptor lambda-chain genes

A

a. T-cell receptor alpha genes

101
Q

Autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED) is caused by a defect in ___________.

A

a transcription factor that regulates tissue-specific gene expression in the thymus

102
Q

Identify which of the following describes how antigen processing and presentation of self antigens by thymic epithelial cells differs from that of antigen-presenting cells in peripheral tissues.

a. Thymic epithelium expresses MHC class I molecules but not MHC class II molecules.
b. Thymic epithelium uses cathepsin L for proteolytic degradation of self proteins.
c. Thymic epithelium expresses MHC class II molecules but not MHC class I molecules.
d. Thymic epithelium expresses transcription repressor protein FoxP3.
e. Thymic epithelium expresses MHC class II molecules but not MHC class I molecules.

A

b. Thymic epithelium uses cathepsin L for proteolytic degradation of self proteins.

103
Q

All of the following types of protein are processed and presented by macrophages in the thymus except _____ proteins.

a. tissue-specific
b. soluble proteins from extracellular fluids
c. ubiquitous proteins
d. proteins made by macrophages
e. proteins derived from other cells that macrophages phagocytose

A

a. tissue-specific

104
Q

Healthy individuals have approximately ____ of CD4 T cells compared with CD8 T cells.

A

twice the number

105
Q

Double-negative thymocytes initiate rearrangement at the _____ locus (loci) before all other T- cell receptor genes.

A

beta, gamma, and delta

106
Q

The function of negative selection of thymocytes in the thymus is to eliminate _____.

A

autoreactive thymocytes

107
Q

Which of the following statements is correct?

a. In adults the mature T-cell repertoire is self-renewing and long-lived and does not require a thymus for the provision of new T cells.
b. T cells and B cells are both short-lived cells and require continual replenishment from primary lymphoid organs.
c. The human thymus is not fully functional until age 30, at which time it begins to shrink and atrophy.
d. In DiGeorge syndrome the bone marrow takes over the function of the thymus and produces mature peripheral T cells.
e. None of the above statements is correct.

A

a. In adults the mature T-cell repertoire is self-renewing and long-lived and does not require a thymus for the provision of new T cells.

108
Q

Individuals with a defective autoimmune regulator gene (AIRE) exhibit ___.

A

autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED)

109
Q

Giulia McGettigan was born full term with a malformed jaw, cleft palate, a ventricular septal defect, and hypocalcemia. Within 48 hours of birth she developed muscle tetany, convulsions, tachypnea, and a systolic murmur. A chest X-ray showed an enlarged heart and the absence of a thymic shadow. Blood tests showed severely depleted levels of CD4 and CD8 T cells; B-cell numbers were low but within normal range. Parathyroid hormone was undetectable. Fluorescence in situ hybridization of the buccal mucosa revealed a small deletion in the long arm of chromosome 22. Giulia failed to thrive and battled chronic diarrhea and opportunistic infections, including oral candidiasis and Pneumocystis jirovecii, the latter infection causing her death. Giulia most probably had which of the following immunodeficiency diseases?

a. AIDS
b. DiGeorge syndrome
c. bare lymphocyte syndrome
d. chronic granulomatous disease
e. hyper IgM syndrome

A

b. DiGeorge syndrome

110
Q

The human thymus begins to degenerate as early as one year after birth. This process is called ______ and is marked by the accumulation of ___ once occupied by thymocytes.

A

involution; fat