Immunologists have made a breakthrough in understanding a rare immune disease called Job's syndrome - so called because its symptoms include recurring outbreaks of abscesses similar to the boils suffered by the eponymous Biblical character. The new research shows that sufferers lack a specific type of white blood cell, leaving them open to repeated attack by certain bacterial and fungal pathogens.
Writing online in Nature, a team led by Daniel Douek describes how sufferers of Job's syndrome, more properly called hyper-IgE syndrome (HIES), are unable to produce cells called TH17 helper cells. These immune cells produce a protein called interleukin-17, and are crucial for protection against invading pathogens.
The discovery ties in with the fact that sufferers are known to have defects in a gene called stat3, which is involved in the differentiation of TH17 cells, a common type of white blood cell, into their various specific types. The lack of this particular component of the immune system explains why patients experience repeated infections by the same pathogens, particularly fungal infection and Staphylococcus bacteria.
Daniel Douek (National Institute of Health, Bethesda MD, USA)
Abstract available online.
(C) Nature press release.
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