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Immunology Lecture 12
Lecture Outlines, Slides, and Scribes
20
Medical
Graduate
08/29/2009

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Cards

Term
On what chromosomes are the genes for kappa, lambda, and heavy chains of Ig located?
Definition

Heavy - Chr. 14

Kappa - Chr. 2

Lambda - Chr. 22

Term
Where are recomination recognition sequences (RSS) located?
Definition

3' to V gene segments

5' to J gene segments

Both sides of D segments

Term
What is Junctional diversity? How does it lead to more variability?
Definition

Junctional diversity - occurs when recombinases leave a few nucleotides from RSS in coding gene region.

 

Diversity/variability occurs when p-nucleotide addition of nucleotide happens to fix hairpin loop left after recombinase cut.

 

Term
What is N-nucleotide addition? Will both heavy and light chains undergo N-nucleotide addition?
Definition

N-nucleotide addition occurs before ligation when terminal deoxynucleotidyl transferase (TdT) adds 1-15 random bases.

 

Only heavy chains undergo N-nucleotide addition, because TdT is expressed early on in B-cell maturation.

Term
At what region does junctional diversity, P-nucleotide addition and N-nucleotide addition occur?
Definition
CDR3 (antigen-binding region for Ig)
Term
True or False. Once RAG is done with its job, it will be inactivated forever.
Definition

False.

 

RAG can be reactivated if a B-cell fails to make a light-chain on the first try

Or if the B cell membrane Ig is self-reactive.

Term
Signals from which molecules deactivate RAG?
Definition
Ig alpha and beta
Term
What makes the surrogate light chain?
Definition
Genes from V pre-B and lambda-5 (aka Pre-B receptor)
Term
What is Burkitt's Lymphoma? What causes it?
Definition

highly-aggressive B-cell lymphoma

 

Cause: tranlocatons of Chr. 8 (protooncogene myc)

to Chr. 14, 2 or 22 (where H, kappa and lambda chains are)

 

Term
What dyscrasia is caused by a chromosome translocation and presents as mature B cells in the blood?
Definition
Chronic lymphocystic leukemia (CLL) is mature B cells (CD5+) or B-1 cells in blood.  Prognosis if no somatic hypermutaition is 3-4years
Term
What dyscrasia is caused by a chromosome translocation and presents as mature B cells in lymph nodes and other tissues?
Definition
Follicular lymphoma
Term
What is multiple myeloma? How is it related to primary amyloidosis?
Definition

Multiple myeloma is a plasma cell malignancy, which produces too much monoclonal antibodies.

 

Primary amyloidosis

Multiple myeloma causes extra light chains in the blood and ECM which forms amyloid plaques. These plaques build up in extracellular spaces in systemic tissues and lead to multiple organ failure.

Term
What causes secondary amyloidosis?
Definition

Chronic inflammatory conditions (i.e. TB)

or familial Mediterranean fever (hereditary)

Term
What is amyloidosis?
Definition
Abnormal deposition of hyaline, proteinceous material laid down extracellularly that result in atrophy, loss of cells and organ failure.
Term
What is Waldenstrom's macroglobulemia?
Definition

plasma cells and lymphocytes secrete too much IgM.

 

Causes primary amyloidosis, visual impairment, neurologic problems, bleeding, hyperviscosity

Term
What can cause local amyloidosis?
Definition

Alzheimer's (amyloid plaques in brain)

type II DM (amyloid in pancreatic islets_

Term
What dyscrasia is caused by a translocation of chromosomes 12:21, and presents as malignant Pre-B cells in blood and bone marrow?
Definition
Acute lymphoblastic leukemia
Term
What dyscrasia involes the Philadelphia chromosome (9:22) bcr/abl translocation and encodes a novel tyrosine kinase?
Definition
Chronic myelogenous leukemia
Term
What is heavy chain disease?
Definition
Malignancy where heavy chain does not associated with light chain. Light chains found in urine as Bence Jones proteins.
Term
Compare B cells and T cells - what is a primitive first-appearing cell? What is considered mature? What are the associated signal molecules?
Definition

Primitive first appearing cell:

- B cell - CD5+ cell

- T cell - gamma/delta T cell

 

Mature cell:

- B cell -  CD5- cell

- T cell - alpha/beta T cell

 

Associated signal molecules

- B cell - Ig alpha/beta

- T cell - CD3

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