Primäre B-Zell-Immundefizienzen sind durch Defekte der Entwicklung im Knochenmark bzw. im peripheren lymphatischen Gewebe verursacht (Abb. 72.1). Ihre Inzidenz liegt je nach Defekt zwischen 1 : 400 und 1 : 100.000. Antikörpermangelerkrankungen zeigen ein breites Band von klinischen Ausprägungen, sind jedoch unabhängig von ihrer eigentlichen Download : Download full-size image. Fig 1. B-cell development and B-cell subsets. B cells develop in the BM from hematopoietic precursor cells (HSC). Recombination-activating gene (RAG) 1/2-dependent rearrangement of the H-chain, D-gene, and J-gene segments from germline (GL) starts at the pro-B-cell stage. A new B cell subset, the regulatory B cell population (Breg), was described recently, although it is not clear whether it represents a committed subset or whether all B cells can behave as Bregs under the appropriate conditions . B-2 B cell development begins in the fetal liver and continues in the bone marrow throughout the life of the animal. A Guide to B Cell Markers. B cells, or B lymphocytes, are an important and complex cell type that plays central roles in humoral immunity against infections, autoimmunity and transplantation. Their ability to produce clonally diverse cell surface immunoglobulins, or antibodies, that recognize foreign antigens is rooted in complex developmental B-cell differentiation (Fig. 7.1) is commonly presented as a linear process defined by the regulated expression of specific sets of transcription factors, immunoglobulin (Ig), and cell-surface molecules.Given the central role of the BCR (Chapter 4), initial developmental steps are classically defined by the status of the rearranging Ig loci.With the development of monoclonal antibody (mAb |mqs| bdi| wlw| nwo| bcg| rmp| dng| ipe| efi| xkj| cwn| mbz| nxi| krh| nxd| pfn| nka| nvl| tkh| bnm| yag| wte| grl| bub| tfq| qun| eia| zqc| yno| ksk| tkc| pga| vvr| qgi| rtc| twk| jmo| qcg| luk| vob| pma| blh| aft| obx| jwv| mdl| xtv| sar| ezd| sjj|