Dendritic Cells(DC) are important players in transplant immunology. Which one of these syndromes is associated with loss of IL-12 producing myeloid dendritic cells?
IPEX syndrome 16%IRF8 syndrome 41%BCG syndrome 33%OPES syndrome 8%
Nice work but slightly tough question. Not many responders. No such syndromes as BCG and OPES( made it up). IPEX syndrome is a T reg FOXP3 deficiency syndrome. Hence, the right answer is IRF8 syndrome. A recent article in NEJM May 2011 talks about description of this syndrome in few cases. Infants were evaluated and looked at genetic analysis. Disseminated infection caused by BCG vaccines is the early manifestation of primary immunodeficiencies - such as SCID. Two distinct disease causing mutations were noted. Both effecting Interferon regulatory factor 8 or IRF8 and leading to the disorder. IRF8 is critical for development of monocytes and dendritic cells and anti mycobacterial immunity. This is an interesting finding.
As we discover more and more of such associations ( FOXP3 deficiency and T regs) and this one now- we can use this information for better understanding immunology and hence transplant medicine benefits. Dendritic cells are antigen presenting cells and knowing that IRF8 is crucial in their functioning, perhaps an inhibitory molecule to IRF8 might be a target for future drug development in transplantation. Lets see where this new disease entity takes us: as everyone knows- its not that simple.
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