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60 Cards in this Set
- Front
- Back
Unproductive light gene rearrange can be replaced
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1) using V & J gene segments not used in prev. rear.
2) Further rear can result in a fnx gene. 3) When 2nd joint made->DNA w/ 1st joint excised |
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Surrogate light chain contains
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1) A 2 protiens
a) /\5 protien > constant region b) VpreB > variable region |
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Pre-B-cell receptor
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1) On cell surface > short time.
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What does the expression of the pre-B-cell receptor generate
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1) Positive signal allowing B cell =/ apoptosis > proceeds to next stage of maturation.
2) u, Iga, and IgB > synth. & retained in E.R |
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What happens when the RAG genes are turned back on?
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(i) Light chain gene rearang. Proceeds
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When fnxal light chain gene is formed?
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1) Light chains are made & assemb. w/ u to form IgM.
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What happens to the IgM
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1) Iga & IgB join it > form mature B-cell receptor
2) Which moves to cell surface |
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The appearance of a fnxal b cell receptor leads to ?
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1) Light chain gene rearrangement
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Cell division initiated by signal through the preBcell receptor results?
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1) In a clone of 30-70 small preB cell
2) All have same H gene 3) Potential to have diff L gene |
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TdT
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1) Terminal deoxynucleotidyl transferase
a) Enzyme that adds N nucleotides at the jnx b/t rear. Gene segments b) expressed in proB cells |
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When is TdT expressed?
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1) When the H chain gene begin to rearrng
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When is TdT not expressed?
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1) In small pre-B cells when light chain gene begin to rearrange
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What is the signifigance of TdT expression
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1) N nucleotides are found in all VD and DJ joints of rearranged human H chain genes
2) But only in half the VJ joint of human L chains |
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when is synthesis of Iga & IgB polypeptides is turned on
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1) pro-B cell stage & continues thru life of B cell
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What is the period “ON” for Iga and IgB consitent with
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1) With the polypeptides being necessary for the cell surface expression of immunoglobulin
2) & for the transduction of signals from pre-B cell and B-cell receptors to the interior of the cell |
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when is Iga and IgB genes turned off?
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1) when An stimulated B cells diff into Ab-secreting plasma cells,
2) and their An receptors are no longer required, a) Ig ceases to apprear on cell surface |
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BtK?
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1) Brutons tyrosine kinase
a) Another signal transduction molecule b) Encoded by a gene on the X chrom c) Essential for B cell maturation |
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Patients who lack a fnx Btk gene have?
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1) Almost no circulating Ab b/c tgheir B cells are blocked at the pre-B cell stagge
2) Results in X-linked agammaglobulinemia |
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X-linked agammaglobulinemia?
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1) Leads to recurrent infections
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many B cell tumors carry?
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1) Chromos translocations > join Ig genes to genes regulating cell growth.’
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transformation of a normal cell into a tumor cell involves?
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1) Series of mutations that release the cell from the normal restraints on its growth.
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translocations?
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1) events that fuse part of a chromosome with another
a) in B cell tumorsthe Ig gene has often b/c joined to a gene involved in the control of cell growth |
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Proto-oncogenes?
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1) Genes that cause cancer when their fnx or expression is perturbed.
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Oncogenes?
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1) The viral gnes responsible for transformation
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Two examples of protooncognes?
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1) Burkitt’s lymphoma
2) BCL2 |
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Burkitt's Lymphoma?
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1) The MYC protooncogne
a) On chromosome 8 (i) translocation to either (a) an Ig heavy-chain gene on chrom 14 (b) a k L chain gene on chrom 2 (c) a /\ L chain gne on chrom 22 |
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BCL2?
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1) Translocation found in b cell tumors
2) Normally prevent premature apoptosis 3) In tumors enable B cells to live longer a) So they accumulate additional mustations that can lead to malignant transformation |
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B-1 Cells?
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1) Subset of human Bcells
2) Arise in early embryonic development a) From stem cell 3) Expresses CD5 (a gp) on cell surface |
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CD5?
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1) Gp on cell surface
2) Otherwise considered a marker for the human T cell lineage |
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Specific characteristics of B-1 Cells?
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1) Little or no IgD on surface
2) Distinctive repertoire of antigen receptors 3) A.k.a CD5 B cells |
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B-1 vs. B-2
When first produced? |
1) Fetus VS after birth
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B-1 vs. B-2
N-regions in VDJ junctions? |
1) Few VS extensive
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B-1 vs. B-2
V-region repertoire? |
1) Restricted VS diverse
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B-1 vs. B-2
Primary location? |
1) Peritoneal & pleural cavities VS 2nd lymph organs
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B-1 vs. B-2
mode of renewal? |
1) self renewing(IL-10) VS replaced from bone marrow
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B-1 vs. B-2
spontaneous production of Ig? |
1) High VS low
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B-1 vs. B-2
isotypes secreted? |
1) IgM>>IgG VS IgG> IgM
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B-1 vs. B-2
response to carbohydrate antigen? |
1) Yes VS Maybe
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B-1 vs. B-2
response to protein antigen? |
1) maybe VS Yes
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B-1 vs. B-2
requirement for Tcell help? |
1) No VS yes
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B-1 vs. B-2
somatic hypermutation? |
1) Low-none VS High
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B-1 vs. B-2
Memory development? |
1) Little or none VS yes
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B-1 cell Tumor?
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1) Chronic lymphocytic leukemia (CLL)
a) A bone marrow transplant from identical sibling is successful treatment |
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Self-reactive immature B cells are altered, eliminated or inactivated by?
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1) Contact with self antigens
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when is a B cell 1st described as an immature B cell?
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1) when it first expresses IgM on its surface.
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Becoming a mature B cell involves?
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1) Emigration from the bone marrow
2) Use of alternative splicing of H chaim mRNA a) To place IgD, as well a IgM on the cell surface. |
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Self-Antigens?
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1) Quality control mechanisms
a) Prevent maturation of B cells whose receptors bind to normal components of the human body |
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If B cells whose receptors bind to normal compoenets of the human body were allowed to mature?
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1) They could make potentially disease-causing antibodies
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kinds of self antigens?
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1) Multivalent
2) Soluble |
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immature B cells that do not encounter a stimulatory self-An?
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1) leave the bone marrow and enter the peripheral circulation, expressing both IgM & IgD on their surfaces
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Multivalent self antigens?
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1) Consists of gp,proteoglycans and glycolipids
2) Go through clonal selection >apoptosis |
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Soluble self anitigen?
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1) Soluble proteins & gp
2) When surface IgM of an immature B cell binds to a soluble self-antigen, > Bcell inactive > Does Not Die 3) Becomes anergic |
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Anergy?
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1) The state in which the B cel matures but does not respond to subsequent exposure to antigen
2) Migrate to periphery > where they express IgD > but remain anergic. |
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Receptor Editing?
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1) A process of assessing the compatibility of receptors produced from successive gene rearrang.
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Steps of receptor Editing?
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1) Ligation of IgM on self-antigen
2) Arrest of B cell development 3) New receptor specifity is now expressed a) If new receptor is still self- reactive > apotosis b) If new receptor is no longer self reac> imm B cell migrates to periphery and matures |
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Clonal deletion?
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1) Apoptosis in receptor editing
a) Occurs within the bone marrow or shortly after cell enters peripheral circulation |
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Ligation of IgM on self antigen?
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1) When dev. B cell produces antigen receptors htat are strongly X linked by multivalent self antigen
2) B cell undergoes develop arrest. |
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Arrest of B development?
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1) and cont. L chain rearran.: low cell surface IgM
2) Amount of IgM on the surface is reduced a) RAG genes are not turned off. |
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new receptor specificity?
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1) cont synthesis of RAG proteins allows the cell to cont L chain gene rear.
a) Leads to a new productive rear. & expression of a new L chain (i) Which combines w/ the previous H chain to form new receptor |
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Primary lymphoid follicles?
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1) Within 2nd lymphoid tiss, B cells congregate in organized structures
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