8.2 B Cell development and the functions of antibodies Flashcards
Name 2 B cell genetic defects
XLA - X linked agammaglobinemia
Hyper IgM Syndrome
What happens in XLA?
mutations in BTK (bruton tyrosine kinase) gene, depleting B cells and hence there are no antibodies
more susceptible to bacterial infections
How is XLA treated?
IV Ig
What happens in Hyper IgM Syndrome?
mutations in CD40 ligand gene
70% X-linked
low levels of IgG, IgA, and IgE
How is Hyper IgM syndrome treated?
IV Ig
What are the 4 main stages of a B cell life cycle?
Proliferation
Screening
Activation
Differentiation
What happens in the proliferation phase of B cell life cycles?
B cell precursor rearranges immunoglobulin genes, generating receptors in the bone marrow
What happens in the screening phase of B cell like cycles?
immature B cell bound to the self cell-surface antigen is removed from the repertoire (negative selection)
What happens in the activation phase of B cell screening?
the mature B cells migrate to the peripheral lymphoid organs and are activated when bound to foreign antigen
How do H-chains undergo rearrangement?
D and J regions
then V rearrangement
How is the success of the H-chain rearrangement checked?
if it can be transiently expressed on the B cell surface in a non-functional manner, it is okey dokey
how is rearrangement of L-chains checked?
expressed as IgM on the B cell surface
What is the hallmark of a mature B cell?
co-expression of IgM and IgD
What are the 2 light chains to choose from?
kappa / lambda
the 2 kappa genes are used first and if this doesn’t work we then try lambda
What is the ratio of kappa to lambda light chains?
2-3 : 1
What does allelic exclusion ensure?
one B cell produces antibody of one specificity
How can we stop further recombination?
preventing Rag expression / function