By Raif Geha, Luigi Notarangelo
Учебник по иммунологии.
One of an important features of the adaptive immune procedure is the construction of antibodies. it truly is expected man or woman could make multiple million various particular antibodies. This striking feat is entire via a fancy genetic application performed via B lymphocytes and their precursors within the bone marrow.
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Extra info for Case studies in immunology : a clinical companion
What other monogenic defects have been described in patients with CVID? Genetic defects in CVID Relative frequency TACI very common BAFF-R extremely rare CD19 extremely rare CD20 extremely rare I COS extremely rare Msh5 to be determined Fig. 5 Monogenetic defects associated with CVID. For most cases of CVI D the gene defect remains unknown. TACI deficiency, BAFF-R deficiency, and mutations in Msh5 do not cause CVID in all affected patients, suggesting that the etiology of most cases of CVID is multifactorial.
Hematopoietic stem cell transplantation (HSCT) from an HLA-identical family donor is the treatment of choice for ADA deficiency. However, this option is available to only 15-20% of the patients. Results of HSCT from HLA mismatched related donors are less satisfactory, with significant mortality, and often incomplete immune reconstitution. The administration of ADA, which is commercially available in a form bound to polyethylene glycol (PEG ADA), clears the noxious metabolites and results in improved immune func tion.
Whereas TI - 1 antigens are inefficient inducers of affinity maturation and memory B cells, TI-2 antigens can induce both IgM and some class-switched responses. The initiation of B-cell class switching usually depends on the interaction of B cells and helper T cells via CD40 and CD40 ligand (see Case 2). Class switching in response to TI antigens is thought to involve other mem bers of the tumor necrosis factor ( T NF) /TNF receptor ( TNFR) family the recently discovered T NF-like proteins BAFF (B-cell activating factor belonging to the TNF family) and APRIL (a proliferation-inducing TNF ligand), and their receptors on B cells.